query: where are the protein complexes associated with the electron transport chain located

pos: Electron transport chain aerobic respiration is molecular oxygen although a variety of acceptors other than oxygen such as sulfate exist in anaerobic respiration. Electron transport chains are used for extracting energy via redox reactions from sunlight in photosynthesis or, such as in the case of the oxidation of sugars, cellular respiration. In eukaryotes, an important electron transport chain is found in the inner mitochondrial membrane where it serves as the site of oxidative phosphorylation through the action of ATP synthase. It is also found in the thylakoid membrane of the chloroplast in photosynthetic eukaryotes. In bacteria, the electron transport chain is located in

pos: Oxidative phosphorylation ATP. In eukaryotes, these redox reactions are carried out by a series of protein complexes within the inner membrane of the cell's mitochondria, whereas, in prokaryotes, these proteins are located in the cells' intermembrane space. These linked sets of proteins are called electron transport chains. In eukaryotes, five main protein complexes are involved, whereas in prokaryotes many different enzymes are present, using a variety of electron donors and acceptors. The energy released by electrons flowing through this electron transport chain is used to transport protons across the inner mitochondrial membrane, in a process called "electron transport". This generates potential energy

pos: BCS1L BCS1L Mitochondrial chaperone BCS1 (BCS1L), also known as BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone (h-BCS1), is a protein that in humans is encoded by the "BCS1L" gene. BCS1L is a chaperone protein involved in the assembly of Ubiquinol Cytochrome c Reductase (complex III), which is located in the inner mitochondrial membrane and is part of the electron transport chain. Mutations in this gene are associated with mitochondrial complex III deficiency (nuclear, 1), GRACILE syndrome, and Bjoernstad syndrome. "BCS1L" is located on the q arm of chromosome 2 in position 35 and has 10 exons. The "BCS1L" gene produces a

pos: ETFDH can be restored by riboflavin, carnitine, or Coenzyme Q10 supplements. The encoded protein interacts with MYH7B, LINC00174, LINC00574, Homeobox protein goosecoid-2, AIRE, OTX1, Keratin-associated protein 13-2, Keratin-associated protein 11-1, TRIM69, Zinc finger protein 581, and COX6B1. ETFDH Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial is an enzyme that in humans is encoded by the "ETFDH" gene. This gene encodes a component of the electron-transfer system in mitochondria and is essential for electron transfer from a number of mitochondrial flavin-containing dehydrogenases to the main respiratory chain. Electron-transferring-flavoprotein dehydrogenase in the inner mitochondrial membrane accepts electrons from electron-transfer flavoprotein which is located in the

pos: PET100 PET100 PET100 homolog is a protein that in humans is encoded by the "PET100" gene. Mitochondrial complex IV, or cytochrome c oxidase, is a large transmembrane protein complex that is part of the respiratory electron transport chain of mitochondria. The small protein encoded by the "PET100" gene plays a role in the biogenesis of mitochondrial complex IV. This protein localizes to the inner mitochondrial membrane and is exposed to the intermembrane space. Mutations in this gene are associated with mitochondrial complex IV deficiency. This gene has a pseudogene on chromosome 3. Alternative splicing results in multiple transcript variants. The "PET100"

pos: BCS1L and R306H. These mutations tend to affect the protein-protein interactions of BCS1L. BCS1L has 11 protein-protein interactions with 8 of them being co-complex interactions. BCS1L has been found to interact with LETM1, DNAJA1, and DDX24. BCS1L Mitochondrial chaperone BCS1 (BCS1L), also known as BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone (h-BCS1), is a protein that in humans is encoded by the "BCS1L" gene. BCS1L is a chaperone protein involved in the assembly of Ubiquinol Cytochrome c Reductase (complex III), which is located in the inner mitochondrial membrane and is part of the electron transport chain. Mutations in this gene are

pos: Beta oxidation Beta oxidation In biochemistry and metabolism, beta-oxidation is the catabolic process by which fatty acid molecules are broken down in the cytosol in prokaryotes and in the mitochondria in eukaryotes to generate acetyl-CoA, which enters the citric acid cycle, and NADH and FADH, which are co-enzymes used in the electron transport chain. It is named as such because the beta carbon of the fatty acid undergoes oxidation to a carbonyl group. Beta-oxidation is primarily facilitated by the mitochondrial trifunctional protein, an enzyme complex associated with the inner mitochondrial membrane, although very long chain fatty acids are oxidized in peroxisomes. The

pos: Beta oxidation disease as inborn errors of metabolism. Beta oxidation In biochemistry and metabolism, beta-oxidation is the catabolic process by which fatty acid molecules are broken down in the cytosol in prokaryotes and in the mitochondria in eukaryotes to generate acetyl-CoA, which enters the citric acid cycle, and NADH and FADH, which are co-enzymes used in the electron transport chain. It is named as such because the beta carbon of the fatty acid undergoes oxidation to a carbonyl group. Beta-oxidation is primarily facilitated by the mitochondrial trifunctional protein, an enzyme complex associated with the inner mitochondrial membrane, although very long chain fatty

neg: MT-ND6 MT-ND6 NADH-ubiquinone oxidoreductase chain 6 is a protein that in humans is encoded by the mitochondrial MT-ND6 gene. The ND6 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Variations in the "MT-ND6" gene are associated with Leigh's syndrome, Leber's hereditary optic neuropathy (LHON) and dystonia. The "MT-ND6" gene is located in human mitochondrial DNA from base pair 14,149 to 14,673. "MT-ND6" is the only protein-coding gene located on the L-strand of the human mitogenome. The encoded protein is 18

neg: NDUFA4 genetically tested for NDUFA4 mutations. NDUFA4 has many protein-protein interactions, including ubiquitin proteins such as ubiquitin C and UBL4A, as well as CUL3 and PARK7. NDUFA4 NDUFA4, mitochondrial complex associated is a protein that in humans is encoded by the NDUFA4 gene. The NDUFA3 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in the NDUFA4 gene are associated with Leigh's syndrome. The NDUFA4 gene is located on the p arm of chromosome 7 at position 21.3 with a

neg: MT-ND4 21 binary protein-protein interactions including 15 co-complex interactions. MT-ND4 appears to interact with SP1, ZNF16, CTCF, GRB2, and ATM. MT-ND4 NADH-ubiquinone oxidoreductase chain 4 is a protein that in humans is encoded by the mitochondrial gene MT-ND4. The ND4 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Variations in the MT-ND4 gene are associated with age-related macular degeneration (AMD), Leber's hereditary optic neuropathy (LHON), mesial temporal lobe epilepsy (MTLE) and cystic fibrosis. The "MT-ND4" gene is located in human

neg: MT-ND6 (NDUFS3) and the ATP-dependent metalloprotease YME1L1. MT-ND6 NADH-ubiquinone oxidoreductase chain 6 is a protein that in humans is encoded by the mitochondrial MT-ND6 gene. The ND6 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Variations in the "MT-ND6" gene are associated with Leigh's syndrome, Leber's hereditary optic neuropathy (LHON) and dystonia. The "MT-ND6" gene is located in human mitochondrial DNA from base pair 14,149 to 14,673. "MT-ND6" is the only protein-coding gene located on the L-strand of the human

neg: Electron transport chain Electron transport chain An electron transport chain (ETC) is a series of complexes that transfer electrons from electron donors to electron acceptors via redox (both reduction and oxidation occurring simultaneously) reactions, and couples this electron transfer with the transfer of protons (H ions) across a membrane. This creates an electrochemical proton gradient that drives the synthesis of adenosine triphosphate (ATP), a molecule that stores energy chemically in the form of highly strained bonds. The molecules of the chain include peptides, enzymes (which are proteins or protein complexes), and others. The final acceptor of electrons in the electron transport chain during

neg: NDUFA4 NDUFA4 NDUFA4, mitochondrial complex associated is a protein that in humans is encoded by the NDUFA4 gene. The NDUFA3 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in the NDUFA4 gene are associated with Leigh's syndrome. The NDUFA4 gene is located on the p arm of chromosome 7 at position 21.3 with a total length of 8,234 base pairs. The NDUFA4 gene produces a 9.4 kDa protein composed of 81 amino acids. NDUFA4 has traditionally been defined as

neg: MT-ND1 MT-ND1 NADH-ubiquinone oxidoreductase chain 1 is a protein that in humans is encoded by the mitochondrial gene MT-ND1. The ND1 protein is a subunit of NADH dehydrogenase, which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Variants of the MT-ND1 gene are associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), Leigh's syndrome (LS), Leber's hereditary optic neuropathy (LHON) and increases in adult BMI. MT-ND1 is located in mitochondrial DNA from base pair 3,307 to 4,262. The MT-ND1 gene produces a 36 kDa protein composed of 318

neg: MT-ND5 MT-ND5 NADH-ubiquinone oxidoreductase chain 5 is a protein that in humans is encoded by the mitochondrial gene MT-ND5. The ND5 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Variations in MT-ND5 are associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) as well as some symptoms of Leigh's syndrome and Leber's hereditary optic neuropathy (LHON). MT-ND5 is located in mitochondrial DNA from base pair 12,337 to 14,148. The MT-ND5 gene produces a 67 kDa protein composed of 603

neg: NDUFAF1 NDUFAF1 Complex I intermediate-associated protein 30, mitochondrial (CIA30), or NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1 (NDUFAF1), is a protein that in humans is encoded by the "NDUFAF1" or "CIA30" gene. The "NDUFAF1" gene encodes a human homolog of a Neurospora crassa protein involved in the assembly of complex I. The NDUFAF1 protein is an assembly factor of NADH dehydrogenase (ubiquinone) also known as complex I, which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Variants of the "NDUFAF1" gene are associated with hypertrophic cardiomyopathy, leukodystrophy,

neg: Mitochondrial matrix forward. The production of ATP also serves as a means of regulation by acting as an inhibitor for Isocitrate dehydrogenase, Pyruvate dehydrogenase, the electron transport chain protein complexes, and ATP syntahase. While ADP acts as an activator. The mitochondria contains its own set of DNA used to produce proteins found in the electron transport chain. The mitochondrial DNA only codes for about thirteen proteins that are used in processing mitochondrial transcripts, ribosomal proteins, ribosomal RNA, transfer RNA, and protein subunits found in the protein complexes of the electron transport chain. Mitochondrial matrix In the mitochondrion, the matrix is the space

neg: MT-ND5 syndrome and/or Leber's hereditary optic neuropathy (LHON) in others. MT-ND5 interacts with Glutamine synthetase (GLUL), LIG4 and YME1L1. MT-ND5 NADH-ubiquinone oxidoreductase chain 5 is a protein that in humans is encoded by the mitochondrial gene MT-ND5. The ND5 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Variations in MT-ND5 are associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) as well as some symptoms of Leigh's syndrome and Leber's hereditary optic neuropathy (LHON). MT-ND5 is located in mitochondrial

neg: MT-ND4 MT-ND4 NADH-ubiquinone oxidoreductase chain 4 is a protein that in humans is encoded by the mitochondrial gene MT-ND4. The ND4 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Variations in the MT-ND4 gene are associated with age-related macular degeneration (AMD), Leber's hereditary optic neuropathy (LHON), mesial temporal lobe epilepsy (MTLE) and cystic fibrosis. The "MT-ND4" gene is located in human mitochondrial DNA from base pair 10,760 to 12,137. An unusual feature of the human "MT-ND4" gene is the 7-nucleotide

neg: MT-ND4L MT-ND4L NADH-ubiquinone oxidoreductase chain 4L is a protein that in humans is encoded by the mitochondrial gene MT-ND4L. The ND4L protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Variants of MT-ND4L are associated with increased BMI in adults and Leber's Hereditary Optic Neuropathy (LHON). The "MT-ND4L" gene is located in human mitochondrial DNA from base pair 10,469 to 10,765. An unusual feature of the human "MT-ND4L" gene is the 7-nucleotide gene overlap of its last three codons (5'-CAA TGC

neg: MT-ND1 BMI in adults and implicated in metabolic disorders including obesity, diabetes and hypertension. MT-ND1 NADH-ubiquinone oxidoreductase chain 1 is a protein that in humans is encoded by the mitochondrial gene MT-ND1. The ND1 protein is a subunit of NADH dehydrogenase, which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Variants of the MT-ND1 gene are associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), Leigh's syndrome (LS), Leber's hereditary optic neuropathy (LHON) and increases in adult BMI. MT-ND1 is located in mitochondrial DNA from base pair 3,307

neg: Mitochondrial matrix that contains the complexes of oxidative phosphorylation. which contains the electron transport chain that is found on the cristae of the inner membrane and consists of four protein complexes and ATP synthase. These complexes are Protein complex I (NADH:Coenzyme Q Oxidoreductase), protein complex II (Succinate:Coenzyme Q Oxidoreductase), protein complex III (Coenzyme Q: Cytochrome C Oxidoreductase), and protein complex IV (Cytochrome C Oxidase). The electron transport chain is responsible for establishing a pH and electrochemical gradient that facilitates the production of ATP through the pumping of protons. The gradient also provides control of the concentration of ions such as Ca driven

neg: NDUFA2 NDUFA2 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 is a protein that in humans is encoded by the "NDUFA2" gene. The NDUFA2 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in the NDUFA2 gene are associated with Leigh's syndrome. The NDUFA2 gene is located on the long (q) arm of chromosome 5 at position 31.2 and it spans 2,422 base pairs. The NDUFA2 gene produces an 11 kDa protein composed of 99 amino acids. NDUFA2 is a

neg: NDUFAF4 NDUFAF4 NADH:ubiquinone oxidoreductase complex assembly factor 4, (NDUFAF4) also known as Hormone-regulated proliferation-associated protein of 20 kDa, (HRPAP20) or C6orf66 is a protein that in humans is encoded by the "NDUFAF4" gene. NDUFAF4 is a mitochondrial assembly protein involved in the assembly of NADH dehydrogenase (ubiquinone) also known as complex I, which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in this gene have been associated with complex I deficiency and infantile mitochondrial encephalomyopathy. Elevations in HRPAP20 have also been implicated in breast cancer. NDUFAF4 is located

neg: NDUFAF3 common signs and symptoms include lactic acidosis, nystagmus, hypotonia, and cerebral lesions. In addition to co-complexes, NDUFAF3 has protein-protein interactions with NDUFAF4 and SNRPA. NDUFAF3 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3, also known as 2P1, E3-3, or C3orf60, is a protein that in humans is encoded by the "NDUFAF3" gene. NDUFAF3 is a mitochondrial assembly protein involved in the assembly of NADH dehydrogenase (ubiquinone) also known as complex I, which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in this gene have been associated

neg: NADH dehydrogenase (ubiquinone), alpha 1 NADH dehydrogenase (ubiquinone), alpha 1 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 is a protein that in humans is encoded by the "NDUFA1" gene. The NDUFA1 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in the NDUFA1 gene are associated with mitochondrial Complex I deficiency. The NDUFA1 gene is located on the long q arm of the X chromosome at position 24 and it spans 5,176 base pairs. The NDUFA1 gene produces an 8.1 kDa protein composed

neg: MT-ND3 MT-ND3 Mitochondrially encoded NADH dehydrogenase 3 is a protein that in humans is encoded by the mitochondrial gene MT-ND3. The ND3 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Variants of MT-ND3 are associated with Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), Leigh's syndrome (LS) and Leber's hereditary optic neuropathy (LHON). MT-ND3 is located in human mitochondrial DNA from base pair 10,059 to 10,404. The MT-ND3 gene produces a 13 kDa protein composed of 115 amino acids. MT-ND3

neg: MT-ND2 MT-ND2 Mitochondrially encoded NADH dehydrogenase 2 is protein that in humans is encoded by the mitochondrial gene MT-ND2 gene. The ND2 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Variants of MT-ND2 are associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), Leigh's syndrome (LS), Leber's hereditary optic neuropathy (LHON) and increases in adult BMI. MT-ND2 is located in mitochondrial DNA from base pair 4,470 to 5,511. The MT-ND2 gene produces a 39 kDa protein composed of 347

neg: Electron transport chain proton gradient). ATP synthase is also readily reversible. Thus ATP can be used to build a proton gradient, which in turn can be used to make NADH. This process of "reverse electron transport" is important in many prokaryotic electron transport chains. In eukaryotes, NADH is the most important electron donor. The associated electron transport chain is NADH → "Complex I" → Q → "Complex III" → cytochrome "c" → "Complex IV" → O where "Complexes I, III" and" IV" are proton pumps, while Q and cytochrome "c" are mobile electron carriers. The electron acceptor is molecular oxygen. In prokaryotes (bacteria

neg: NDUFAF2 NDUFAF2 NADH:ubiquinone oxidoreductase complex assembly factor 2 (NDUFAF2), also known as B17.2L or NDUFA12L is a protein that in humans is encoded by the "NDUFAF2," or "B17.2L," gene. The NDUFAF2 protein is a chaperone involved in the assembly of NADH dehydrogenase (ubiquinone) also known as complex I, which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in this gene have been associated with progressive encephalopathy and Leigh disease resulting from mitochondrial complex I deficiency. "NDUFAF2" is located on the q arm of chromosome 5 in position 12.1.

neg: NDUFAF7 NDUFAF7 Protein arginine methyltransferase NDUFAF7, mitochondrial, also known as NADH:ubiquinone oxidoreductase complex assembly factor 7 (NDUFAF7), MidA, C2orf56, or PRO1853, is a protein that in humans is encoded by the "NDUFAF7" gene. NDUFAF7 is a methyltransferase mitochondrial assembly enzyme involved in the assembly and stabilization of NADH dehydrogenase (ubiquinone) also known as complex I, which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in "NDUFAF7" have been associated with pathologic myopia and complex I deficiency. "NDUFAF7" is located on the p arm of chromosome 2 in position

neg: Cytochrome b Cytochrome b Cytochrome b is a protein found in the mitochondria of eukaryotic cells. It functions as part of the electron transport chain and is the main subunit of transmembrane cytochrome bc1 and b6f complexes. In the mitochondrion of eukaryotes and in aerobic prokaryotes, cytochrome b is a component of respiratory chain complex III () — also known as the bc1 complex or ubiquinol-cytochrome c reductase. In plant chloroplasts and cyanobacteria, there is an analogous protein, cytochrome b6, a component of the plastoquinone-plastocyanin reductase (), also known as the b6f complex. These complexes are involved in electron transport, the pumping

neg: NDUFA11 NDUFA11 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 is an enzyme that in humans is encoded by the NDUFA11 gene. The NDUFA11 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain Mutations in subunits of NADH dehydrogenase (ubiquinone), also known as Complex I, frequently lead to complex neurodegenerative diseases such as Leigh's syndrome. Mutations in this gene are associated with severe mitochondrial complex I deficiency. The NDUFA11 gene is located on the p arm of chromosome 19 in position

neg: NDUFS8 NDUFS8 NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial also known as NADH-ubiquinone oxidoreductase 23 kDa subunit, Complex I-23kD (CI-23kD), or TYKY subunit is an enzyme that in humans is encoded by the "NDUFS8" gene. The NDUFS8 protein is a subunit of NADH dehydrogenase (ubiquinone) also known as Complex I, which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in this gene have been associated with Leigh syndrome. NDUFS8 is located on the q arm of chromosome 11 in position 13.2. The NDUFS8 gene produces a 23.7 kDa

neg: NDUFAF3 NDUFAF3 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3, also known as 2P1, E3-3, or C3orf60, is a protein that in humans is encoded by the "NDUFAF3" gene. NDUFAF3 is a mitochondrial assembly protein involved in the assembly of NADH dehydrogenase (ubiquinone) also known as complex I, which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in this gene have been associated severe complex I deficiency and Leigh syndrome. NDUFAF3 is located on the p arm of chromosome 3 in position 21.31 and has 7 exons.

neg: Mitochondrion of FADH) that are a source of electrons for the "electron transport chain", and a molecule of GTP (that is readily converted to an ATP). The redox energy from NADH and FADH is transferred to oxygen (O) in several steps via the electron transport chain. These energy-rich molecules are produced within the matrix via the citric acid cycle but are also produced in the cytoplasm by glycolysis. Reducing equivalents from the cytoplasm can be imported via the malate-aspartate shuttle system of antiporter proteins or feed into the electron transport chain using a glycerol phosphate shuttle. Protein complexes in the inner

neg: NDUFA10 NDUFA10 appears to interact with RAB8A. NDUFA10 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10 is an enzyme that in humans is encoded by the NDUFA10 gene. The NDUFA10 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in subunits of NADH dehydrogenase (ubiquinone), also known as Complex I, frequently lead to complex neurodegenerative diseases such as Leigh's syndrome. Furthermore, reduced NDUFA10 expression levels due to FOXM1-directed hypermethylation are associated with human squamous cell carcinoma and may be related

neg: NDUFA10 NDUFA10 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10 is an enzyme that in humans is encoded by the NDUFA10 gene. The NDUFA10 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in subunits of NADH dehydrogenase (ubiquinone), also known as Complex I, frequently lead to complex neurodegenerative diseases such as Leigh's syndrome. Furthermore, reduced NDUFA10 expression levels due to FOXM1-directed hypermethylation are associated with human squamous cell carcinoma and may be related to other forms of cancer. The

neg: NDUFB5 NDUFB5 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5, 16kDa is a protein that in humans is encoded by the NDUFB5 gene. The NDUFB5 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. The NDUFB5 gene, located on the q arm of chromosome 3 in position 26.33, is 19,713 base pairs long. The NDUFB5 protein weighs 21.7 kDa and is composed of 189 amino acids. NDUFB5 is a subunit of the enzyme NADH dehydrogenase (ubiquinone), the largest of the respiratory complexes.

neg: NDUFB4 NDUFB4 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 4, 15kDa is a protein that in humans is encoded by the NDUFB4 gene. The NDUFB4 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. The NDUFB4 gene, located on the q arm of chromosome 3 in position 13.33, is 6,130 base pairs long. The NDUFB4 protein weighs 15 kDa and is composed of 129 amino acids. NDUFB4 is a subunit of the enzyme NADH dehydrogenase (ubiquinone), the largest of the respiratory complexes.

neg: NDUFA4L2 NDUFA4L2 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4-like 2 is a protein that in humans is encoded by the NDUFA4L2 gene. The NDUFA4L2 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. The NDUFA4L2 gene is located on the long q arm of chromosome 12 at position 13.3 and it spans 5,860 base pairs. NDUFA4L2 is a subunit of the enzyme NADH dehydrogenase (ubiquinone), the largest of the respiratory complexes. The structure is L-shaped with a long, hydrophobic transmembrane domain

neg: Deinococcus frigens 3987 are protein-coding. The JGI IMG database shows genes which are found within "D. frigens" and associated with metabolic pathways found in the KEGG database. For carbohydrate metabolism, the genome of "D. frigens" contains genes necessary for metabolism of fructose to glucose, galactose to glucose, the entirety of the glycolysis pathway, pyruvate metabolism, TCA cycle, gluconeogenesis, the pentose phosphate pathway. Additionally, the genome includes genes necessary for extracellular nitrate and nitrite transport, assimilatory reduction of nitrite to ammonia, assimilatory reduction of nitrate to nitrite, and sulfite reduction. The electron transport chain of "D. frigens" is made up of five complexes:

neg: FOXRED1 FOXRED1 FAD-dependent oxidoreductase domain-containing protein 1 (FOXRED1), also known as H17, or FP634 is an enzyme that in humans is encoded by the "FOXRED1" gene. FOXRED1 is an oxidoreductase and complex I-specific molecular chaperone involved in the assembly and stabilization of NADH dehydrogenase (ubiquinone) also known as complex I, which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in "FOXRED1" have been associated with Leigh syndrome and infantile-onset mitochondrial encephalopathy. "FOXRED1" is located on the q arm of chromosome 11 in position 14.2 and has 12 exons.

neg: NDUFA9 NDUFA9 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9 is an enzyme that in humans is encoded by the NDUFA9 gene. The NDUFA9 protein is a subunit of (Complex I of the electron transport chain), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in NADH dehydrogenase (ubiquinone), also known as Complex I, frequently lead to complex neurodegenerative diseases such as Leigh's syndrome. In the case of NDUFA9, a mutation to the MT-ND3 gene might interrupt their interaction and formation of subcomplexes, compromising Complex I function and

neg: NDUFA5 NDUFA5 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 is an enzyme that in humans is encoded by the "NDUFA5" gene. The NDUFA5 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. The NDUFA5 gene is located on the q arm of chromosome 7 and it spans 64,655 base pairs. The gene produces a 13.5 kDa protein composed of 116 amino acids. NDUFA5 is a subunit of the enzyme NADH dehydrogenase (ubiquinone), the largest of the respiratory complexes. The structure

neg: NDUFA7 NDUFA7 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 is an enzyme that in humans is encoded by the NDUFA7 gene. The NDUFA7 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. The NDUFA7 gene is located on the p arm of chromosome 19 in position 13.2 and spans 12,618 base pairs. The gene produces a 12.5 kDa protein composed of 113 amino acids. NDUFA7 is a subunit of the enzyme NADH dehydrogenase (ubiquinone), the largest of the respiratory complexes.

neg: NDUFA8 NDUFA8 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 is an enzyme that in humans is encoded by the NDUFA8 gene. The NDUFA8 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. The NDUFA8 gene is located on the q arm of chromosome 9 in position 33.2 and spans 27,354 base pairs. The gene produces a 20 kDa protein composed of 172 amino acids. NDUFA8 is a subunit of the enzyme NADH dehydrogenase (ubiquinone), the largest of the respiratory complexes.

neg: NDUFA6 NDUFA6 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 is an enzyme that in humans is encoded by the NDUFA6 gene. The NDUFA6 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. The NDUFA6 gene is located on the q arm of chromosome 22 in position 13.2 and spans 5,359 base pairs. The gene produces an 18 kDa protein composed of 154 amino acids. NDUFA6 is a subunit of the enzyme NADH dehydrogenase (ubiquinone), the largest of the respiratory complexes.

neg: NDUFA13 NDUFA13 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 is an enzyme that in humans is encoded by the "NDUFA13" gene. The NDUFA13 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. The NDUFA13 gene is located on the p arm of chromosome 19 in position 13.2 and spans 11,995 base pairs. The gene produces a 17 kDa protein composed of 144 amino acids. NDUFA13 is a subunit of the enzyme NADH dehydrogenase (ubiquinone), the largest of the respiratory complexes.

neg: NDUFA5 protein-protein interactions, such as ubiquitin C and with members of the NADH dehydrogenase [ubiquinone] 1 beta subcomplex, including NDUFB1, NDUFB9 and NDUFB10. NDUFA5 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 is an enzyme that in humans is encoded by the "NDUFA5" gene. The NDUFA5 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. The NDUFA5 gene is located on the q arm of chromosome 7 and it spans 64,655 base pairs. The gene produces a 13.5 kDa protein composed

neg: Light-dependent reactions the production of NADPH. Cyclic phosphorylation is important to create ATP and maintain NADPH in the right proportion for the light-independent reactions. The net-reaction of all light-dependent reactions in oxygenic photosynthesis is: The two photosystems are protein complexes that absorb photons and are able to use this energy to create an electron transport chain. Photosystem I and II are very similar in structure and function. They use special proteins, called light-harvesting complexes, to absorb the photons with very high effectiveness. If a special pigment molecule in a photosynthetic reaction center absorbs a photon, an electron in this pigment attains the

neg: NDUFAB1 NDUFAB1 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5, 16kDa is a protein that in humans is encoded by the NDUFAB1 gene. The NDUFAB1 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. The NDUFAB1 gene is located on the p arm of chromosome 16 at position 12.2 and it spans 15,327 base pairs. The NDUFAB1 gene produces a 17.4 kDa protein composed of 156 amino acids. NDUFAB1 is a subunit of the enzyme NADH dehydrogenase (ubiquinone), the largest of the

neg: NDUFA3 NDUFA3 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 is a protein that in humans is encoded by the "NDUFA3" gene. The NDUFA3 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. The NDUFA3 gene is located on the q arm of chromosome 19 at position 13.42, and it has a total span of 4,123 base pairs. The NDUFA3 gene produces an 9.3 kDa protein composed of 84 amino acids. NDUFA3 is a subunit of the enzyme NADH dehydrogenase (ubiquinone),

neg: Tafazzin protein transport within cells. During apoptosis and similar processes, CL is known to act as a platform for proteins and other machinery involved. Cardiolipin has been shown to assist in energy production of the mitochondria. Several proteins in the mitochondrial respiratory chain require CL for optimal function. CL has been found to be involved in the stabilization of each respiratory chain complex, enabling efficient electron transport. CL assists in forming super-complexes with proteins localized in the inner mitochondrial matrix, which include the ATP/ADP translocase, pyruvate carrier, carnitine carrier, and all of the respiratory chain complexes (I, III,IV, V). CL also

neg: NDUFS7 NDUFS7 NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial, also knowns as NADH-ubiquinone oxidoreductase 20 kDa subunit, Complex I-20kD (CI-20kD), or PSST subunit is an enzyme that in humans is encoded by the "NDUFS7" gene. The NDUFS7 protein is a subunit of NADH dehydrogenase (ubiquinone) also known as Complex I, which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. The NDUFS7 gene is located on the p arm of chromosome 19 in position 13.3. The NDUFS7 gene produces a 25 kDa protein composed of 238 amino acids. The PSST

neg: Electron transport chain of protons across a membrane, producing a proton gradient. The proton gradient is used to produce useful work. About 30 work units are produced per electron transport. Electron transport chain An electron transport chain (ETC) is a series of complexes that transfer electrons from electron donors to electron acceptors via redox (both reduction and oxidation occurring simultaneously) reactions, and couples this electron transfer with the transfer of protons (H ions) across a membrane. This creates an electrochemical proton gradient that drives the synthesis of adenosine triphosphate (ATP), a molecule that stores energy chemically in the form of highly strained bonds.

neg: NDUFAB1 treatments. Moreover, a study to identify small molecule drug targets for Tetralogy of Fallot (TOF), a congenital malformation of the heart, found the "NDUFAB1" gene to be a major hub gene of differentially expressed genes in TOF. NDUFAB1 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5, 16kDa is a protein that in humans is encoded by the NDUFAB1 gene. The NDUFAB1 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. The NDUFAB1 gene is located on the p arm of chromosome

neg: NDUFB3 NDUFB3 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 3, 12kDa is a protein that in humans is encoded by the NDUFB3 gene. NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 3, 12kDa is an accessory subunit of the NADH dehydrogenase (ubiquinone) complex, located in the mitochondrial inner membrane. It is also known as Complex I and is the largest of the five complexes of the electron transport chain. Mutations in this gene contribute to mitochondrial complex I deficiency. The NDUFB3 gene, located on the q arm of chromosome 2 in position 31.3, is 14,012 base pairs long. The NDUFB3 protein weighs 11.4 kDa

neg: NDUFB6 NDUFB6 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6, also known as complex I-B17, is a protein that in humans is encoded by the "NDUFB6" gene. NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 6, is an accessory subunit of the NADH dehydrogenase (ubiquinone) complex, located in the mitochondrial inner membrane. It is also known as Complex I and is the largest of the five complexes of the electron transport chain. The NDUFB6 gene is located on the p arm of chromosome 9 in position 21.1 and is 19,659 base pairs long. The NDUFB6 protein weighs 15.5 kDa and is composed

neg: NDUFB3 defect in the respiratory chain. NDUFB3 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 3, 12kDa is a protein that in humans is encoded by the NDUFB3 gene. NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 3, 12kDa is an accessory subunit of the NADH dehydrogenase (ubiquinone) complex, located in the mitochondrial inner membrane. It is also known as Complex I and is the largest of the five complexes of the electron transport chain. Mutations in this gene contribute to mitochondrial complex I deficiency. The NDUFB3 gene, located on the q arm of chromosome 2 in position 31.3, is 14,012 base pairs long. The

neg: Electron transport chain can synthesize different transmembrane complexes and produce different electron transport chains in their cell membranes. Bacteria select their electron transport chains from a DNA library containing multiple possible dehydrogenases, terminal oxidases and terminal reductases. The situation is often summarized by saying that electron transport chains in bacteria are "branched", "modular", and "inducible". In oxidative phosphorylation, electrons are transferred from a low-energy electron donor (e.g., NADH) to an acceptor (e.g., O) through an electron transport chain. In photophosphorylation, the energy of sunlight is used to "create" a high-energy electron donor and an electron acceptor. Electrons are then transferred from the donor

neg: Vesicular transport adaptor protein Vesicular transport adaptor protein Vesicular transport adaptor proteins are proteins involved in forming complexes that function in the trafficking of molecules from one subcellular location to another. These complexes concentrate the correct cargo molecules in vesicles that bud or extrude off of one organelle and travel to another location, where the cargo is delivered. While some of the details of how these adaptor proteins achieve their trafficking specificity has been worked out, there is still much to be learned. There are several human disorders associated with defects in components of these complexes including Alzheimer's and Parkinson's diseases. Most of the

neg: Photosystem I those of cyanobacteria, algae, and higher plants are not the same, however there are many analogous functions and similar structures. Three main features are similar between the different photosystems. First, redox potential is negative enough to reduce ferredoxin. Next, the electron-accepting reaction centers include iron–sulfur proteins. Last, redox centres in complexes of both photosystems are constructed upon a protein subunit dimer. The photosystem of green sulfur bacteria even contains all of the same cofactors of the electron transport chain in PSI. The number and degree of similarities between the two photosystems strongly indicates that PSI is derived from the analogous

neg: COX6B1 been linked to complex IV deficiency with encephalomyopathy, hydrocephalus, and hypertrophic cardiomyopathy. COX6B1 has been shown to have 548 binary protein-protein interactions including 547 co-complex interactions. COX6B1 Cytochrome c oxidase subunit 6B1 is an enzyme that in humans is encoded by the "COX6B1" gene. Cytochrome "c" oxidase 6B1 is a subunit of the cytochrome "c" oxidase complex, also known as Complex IV, the last enzyme in the mitochondrial electron transport chain. Mutations of the COX6B1 gene are associated with severe infantile encephalomyopathy and mitochondrial complex IV deficiency (MT-C4D). The COX6B1 gene, located on the q arm of chromosome 19 in

neg: NDUFB10 NDUFB10 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 is an enzyme that in humans is encoded by the "NDUFB10" gene. NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 10 is an accessory subunit of the NADH dehydrogenase (ubiquinone) complex, located in the mitochondrial inner membrane. It is also known as Complex I and is the largest of the five complexes of the electron transport chain. The NDUFB10 gene is located on the p arm of chromosome 16 in position 13.3 and is 2,459 base pairs long. The NDUFB10 protein weighs 21 kDa and is composed of 172 amino acids. NDUFB10

neg: NDUFB7 NDUFB7 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7, also known as complex I-B18, is an enzyme that in humans is encoded by the "NDUFB7" gene. NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 7 is an accessory subunit of the NADH dehydrogenase (ubiquinone) complex, located in the mitochondrial inner membrane. It is also known as Complex I and is the largest of the five complexes of the electron transport chain. The NDUFB7 gene is located on the p arm of chromosome 19 in position 13.12 and is 6,000 base pairs long. The NDUFB7 protein weighs 16.4 kDa and is composed

neg: NDUFB2 NDUFB2 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial is an enzyme that in humans is encoded by the "NDUFB2" gene. NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 2, 8kDa is an accessory subunit of the NADH dehydrogenase (ubiquinone) complex, located in the mitochondrial inner membrane. It is also known as Complex I and is the largest of the five complexes of the electron transport chain. The NDUFB2 gene, located on the q arm of chromosome 7 in position 34, is 9,966 base pairs long and is composed of 4 exons. The NDUFB2 protein weighs 12 kDa and is composed

neg: NDUFB1 NDUFB1 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 is an enzyme that in humans is encoded by the "NDUFB1" gene. NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 1, 7kDa is an accessory subunit of the NADH dehydrogenase (ubiquinone) complex, located in the mitochondrial inner membrane. It is also known as Complex I and is the largest of the five complexes of the electron transport chain. The NDUFB1 gene, located on the q arm of chromosome 14 in position 32.12, is 5,687 base pairs long. The NDUFB1 protein weighs 7 kDa and is composed of 58 amino acids. NDUFB1 is a

neg: NDUFB9 NDUFB9 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 is an enzyme that in humans is encoded by the "NDUFB9" gene. NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 9 is an accessory subunit of the NADH dehydrogenase (ubiquinone) complex, located in the mitochondrial inner membrane. It is also known as Complex I and is the largest of the five complexes of the electron transport chain. The NDUFB9 gene is located on the q arm of chromosome 8 in position 13.3 and is 10,884 base pairs long. The NDUFB9 protein weighs 22 kDa and is composed of 179 amino acids. NDUFB9

neg: NDUFB8 NDUFB8 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial is an enzyme that in humans is encoded by the "NDUFB8" gene. NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 8 is an accessory subunit of the NADH dehydrogenase (ubiquinone) complex, located in the mitochondrial inner membrane. It is also known as Complex I and is the largest of the five complexes of the electron transport chain. The NDUFB8 gene is located on the q arm of chromosome 10 in position 24.31 and is 6,194 base pairs long. The NDUFB8 protein weighs 22 kDa and is composed of 186 amino acids.

neg: NDUFA12 NDUFA12 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 is an enzyme that in humans is encoded by the "NDUFA12" gene. The NDUFA12 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in subunits of NADH dehydrogenase (ubiquinone), also known as Complex I, frequently lead to complex neurodegenerative diseases such as Leigh's syndrome that result from mitochondrial complex I deficiency. The NDUFA12 gene is located on the q arm of chromosome 12 in position 22 and spans 32,386 base

neg: Pyocyanin a wide range of cellular components and pathways. Pathways which are affected by pyocyanin include the electron transport chain, vesicular transport, and cell growth. An enhanced susceptibility to pyocyanin is seen in cells which have mutation in certain proteins or complexes. Mutations in genes affecting V-ATPase synthesis and assembly, vesicle transport machinery, protein sorting machinery all confer an increased sensitivity to pyocyanin which further enhances the effects on cystic fibrosis on the patient. Vacuolar- ATPase in yeast cells is a particularly potent target as it is the main non-mitochondrial producer of ATP but also has numerous other functions such as

neg: Cytochrome c Cytochrome c The cytochrome complex, or cyt "c" is a small hemeprotein found loosely associated with the inner membrane of the mitochondrion. It belongs to the cytochrome c family of proteins. Cytochrome c is highly water-soluble, unlike other cytochromes, and is an essential component of the electron transport chain, where it carries one electron. It is capable of undergoing oxidation and reduction, but does not bind oxygen. It transfers electrons between Complexes III (Coenzyme Q – Cyt C reductase) and IV (Cyt C oxidase). In humans, cytochrome c is encoded by the "CYCS" gene. Cytochrome c is a highly conserved

neg: NDUFB11 NDUFB11 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial (NADH-ubiquinone oxidoreductase ESSS subunit) is an enzyme that in humans is encoded by the "NDUFB11" gene. NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 11 is an accessory subunit of the NADH dehydrogenase (ubiquinone) complex, located in the mitochondrial inner membrane. It is also known as Complex I and is the largest of the five complexes of the electron transport chain. "NDUFB11" mutations have been associated with linear skin defects with multiple congenital anomalies 3 and mitochondrial complex I deficiency. The NDUFB11 gene is located on the p arm of chromosome

neg: Cytochrome b the anti-malarial drug atovaquone. Human genes encoding cytochrome b proteins include: Cytochrome b Cytochrome b is a protein found in the mitochondria of eukaryotic cells. It functions as part of the electron transport chain and is the main subunit of transmembrane cytochrome bc1 and b6f complexes. In the mitochondrion of eukaryotes and in aerobic prokaryotes, cytochrome b is a component of respiratory chain complex III () — also known as the bc1 complex or ubiquinol-cytochrome c reductase. In plant chloroplasts and cyanobacteria, there is an analogous protein, cytochrome b6, a component of the plastoquinone-plastocyanin reductase (), also known as the

neg: Tetratricopeptide repeat protein 39B another create a right-handed helical structure with an amphipathic channel which could possibly accommodate the complementary region of a target protein. Most TPR-containing proteins are associated with multiprotein complexes, and there is extensive evidence indicating that TPR motifs are important to the functioning of chaperone, cell-cycle, transcription, and protein transport complexes. Two more TPR domains are found in the TTC39B protein: TPR1 which spans from amino acids 393 to 426 (34 amino acids long) and TPR2 which spans from amino acids 626 to 659 (also 34 amino acids long). TTC39B contains three transmembrane regions, all located within the DUF3808 region.

neg: Mitochondrial matrix The oxidation of NADH and FADH produces GTP from succinyl-CoA synthetase. NADH and FADH2 are produced in the matrix or transported in through porin and transport proteins in order to undergo oxidation through oxidative phosphorylation. NADH and FADH2 undergo oxidation in the electron transport chain by transferring a electrons to regenerate NADand FAD. Protons are pulled into the intermembrane space by the energy of the electrons going through the electron transport chain. Four electrons are finally accepted by oxygen in the matrix to complete the electron transport chain. The protons return to the mitochondrial matrix through the protein ATP synthase.The

neg: Electrochemical gradient animal cell has a transmembrane electrical potential of -50 mV to -70 mV inside the cell relative to the outside. Electrochemical gradients also play a role in establishing proton gradients in oxidative phosphorylation in mitochondria. The final step of cellular respiration is the electron transport chain. Four complexes embedded in the inner membrane of the mitochondrion make up the electron transport chain. However, only complexes I, III, and IV pump protons from the matrix to the intermembrane space (IMS). In total, there are ten protons translocated from the matrix to the IMS which generates an electrochemical potential of more than

neg: Electron transport chain to two molecules of cytochrome "c", a water-soluble electron carrier located within the intermembrane space. The two other electrons sequentially pass across the protein to the Q site where the quinone part of ubiquinone is reduced to quinol. A proton gradient is formed by one quinol (<chem>2H+2e-</chem>) oxidations at the Q site to form one quinone (<chem>2H+2e-</chem>) at the Q site. (in total four protons are translocated: two protons reduce quinone to quinol and two protons are released from two ubiquinol molecules). When electron transfer is reduced (by a high membrane potential or respiratory inhibitors such as antimycin A), Complex

neg: Electron transport chain ions to reduce molecular oxygen to water. Four membrane-bound complexes have been identified in mitochondria. Each is an extremely complex transmembrane structure that is embedded in the inner membrane. Three of them are proton pumps. The structures are electrically connected by lipid-soluble electron carriers and water-soluble electron carriers. The overall electron transport chain: In "Complex I" (NADH:ubiquinone oxidoreductase, NADH-CoQ reductase, or NADH dehydrogenase; ), two electrons are removed from NADH and ultimately transferred to a lipid-soluble carrier, ubiquinone (UQ). The reduced product, ubiquinol (UQH), freely diffuses within the membrane, and Complex I translocates four protons (H) across the membrane, thus

neg: Protein complex proteins assemble in a way that mimics evolution. That is, an intermediate in the assembly process is present in the complex’s evolutionary history. The opposite phenomenon is observed in heteromultimeric complexes, where gene fusion occurs in a manner that preserves the original assembly pathway. Protein complex A protein complex or multiprotein complex is a group of two or more associated polypeptide chains. Different polypeptide chains may have different functions. This is distinct from a multienzyme complex, in which multiple catalytic domains are found in a single polypeptide chain. Protein complexes are a form of quaternary structure. Proteins in a protein

neg: COX6A1 COX6A1 Cytochrome c oxidase subunit 6A1, mitochondrial is an protein that in humans is encoded by the "COX6A1" gene. Cytochrome c oxidase 6A1 is a subunit of the cytochrome "c" oxidase complex, also known as Complex IV, the last enzyme in the mitochondrial electron transport chain. A mutation of the COX6A1 gene is associated with a recessive axonal or mixed form of Charcot-Marie-Tooth disease. The COX6A1 gene, located on the q arm of chromosome 12 in position 24.2, contains 3 exons and is 2,653 base pairs in length. The COX6A1 protein weighs 12 kDa and is composed of 109 amino

neg: Cardiolipin to have a lamellar-to-hexagonal (L-H) phase transition. And it is believed to have a close connection with membrane fusion. The enzyme cytochrome c oxidase or Complex IV is a large transmembrane protein complex found in bacteria and the mitochondrion. It is the last enzyme in the respiratory electron transport chain of mitochondria (or bacteria) located in the mitochondrial (or bacterial) membrane. It receives an electron from each of four cytochrome c molecules, and transfers them to one oxygen molecule, converting molecular oxygen to two molecules of water. Complex IV has been shown to require two associated CL molecules in order

neg: COX6B1 COX6B1 Cytochrome c oxidase subunit 6B1 is an enzyme that in humans is encoded by the "COX6B1" gene. Cytochrome "c" oxidase 6B1 is a subunit of the cytochrome "c" oxidase complex, also known as Complex IV, the last enzyme in the mitochondrial electron transport chain. Mutations of the COX6B1 gene are associated with severe infantile encephalomyopathy and mitochondrial complex IV deficiency (MT-C4D). The COX6B1 gene, located on the q arm of chromosome 19 in position 13.1, contains 4 exons and is 10,562 base pairs in length. The COX6B1 protein weighs 10 kDa and is composed of 86 amino acids. The

neg: SDHAF2 cofactor of flavin adenine dinucleotide (FAD). The structure represents a five-helix bundle with a region of well-defined conserved surface residues. This conserved region includes a negatively charged periphery and a positively charged surface, and a patch that is hydrophobic. The region is located in α-helices I, II, and the connecting band. The "SDHAF2" gene encodes a mitochondrial protein associated with the succinate dehydrogenase (SDH) complex (mitochondrial complex II) in the mitochondrial respiratory chain, which plays essential roles in both the electron transport chain and the Krebs(tricarboxylic acid) cycle. SDHAF2 is integral in the proper function of the SDH complex, mainly

neg: Protein complex Protein complex A protein complex or multiprotein complex is a group of two or more associated polypeptide chains. Different polypeptide chains may have different functions. This is distinct from a multienzyme complex, in which multiple catalytic domains are found in a single polypeptide chain. Protein complexes are a form of quaternary structure. Proteins in a protein complex are linked by non-covalent protein–protein interactions, and different protein complexes have different degrees of stability over time. These complexes are a cornerstone of many (if not most) biological processes and together they form various types of molecular machinery that perform a vast array

neg: NDUFS6 failure. NDUFS6 NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial is an enzyme that in humans is encoded by the "NDUFS6" gene. The multisubunit NADH:ubiquinone oxidoreductase (complex I) is the first enzyme complex in the electron transport chain of mitochondria. The iron-sulfur protein (IP) fraction is made up of 7 subunits, including NDUFS6. Mutations in the "NDUFS6" gene are associated with mitochondrial Complex I deficiency, and are inherited in an autosomal recessive manner. This deficiency is the most common enzymatic defect of the oxidative phosphorylation disorders. Mitochondrial complex I deficiency shows extreme genetic heterogeneity and can be caused by mutation in

neg: ATP5D protein has been known to resemble a 'lollipop' structure due to the attachment of the F1 catalytic unit to the mitochondrial inner membrane by the F0 unit. This gene encodes a subunit of the mitochondrial ATP synthase (Complex V) of the mitochondrial respiratory chain, which is necessary for the catalysis of ATP synthesis. Utilizing an electrochemical gradient of protons produced by electron transport complexes of the respiratory chain, the synthase converts ADP into ATP across the inner membrane during oxidative phosphorylation. F-type ATPases consist of two structural domains, F1 and F0, that contribute to the catalysis. The F1 domain contains

neg: Clathrin adaptor protein Clathrin adaptor protein Clathrin adaptor proteins, also known as adaptins, are vesicular transport adaptor proteins associated with clathrin. These proteins are synthesized in the ribosomes, processed in the endoplasmic reticulum and transported from the Golgi apparatus to the trans-Golgi network, and from there via small carrier vesicles to their final destination compartment. The association between adaptins and clathrin are important for vesicular cargo selection and transporting. Clathrin coats contain both clathrin (acts as a scaffold) and adaptor complexes that link clathrin to receptors in coated vesicles. Clathrin-associated protein complexes are believed to interact with the cytoplasmic tails of membrane proteins,

neg: NDUFS6 NDUFS6 NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial is an enzyme that in humans is encoded by the "NDUFS6" gene. The multisubunit NADH:ubiquinone oxidoreductase (complex I) is the first enzyme complex in the electron transport chain of mitochondria. The iron-sulfur protein (IP) fraction is made up of 7 subunits, including NDUFS6. Mutations in the "NDUFS6" gene are associated with mitochondrial Complex I deficiency, and are inherited in an autosomal recessive manner. This deficiency is the most common enzymatic defect of the oxidative phosphorylation disorders. Mitochondrial complex I deficiency shows extreme genetic heterogeneity and can be caused by mutation in nuclear-encoded

neg: Oxidative phosphorylation to stresses such as cold, reactive oxygen species, and infection by pathogens, as well as other factors that inhibit the full electron transport chain. Alternative pathways might, therefore, enhance an organisms' resistance to injury, by reducing oxidative stress. The original model for how the respiratory chain complexes are organized was that they diffuse freely and independently in the mitochondrial membrane. However, recent data suggest that the complexes might form higher-order structures called supercomplexes or "respirasomes". In this model, the various complexes exist as organized sets of interacting enzymes. These associations might allow channeling of substrates between the various enzyme complexes,

neg: COQ6 COQ6 Coenzyme Q6 monooxygenase is a protein that in humans is encoded by the COQ6 gene. The protein encoded by this gene belongs to the ubiH/COQ6 family. It is an evolutionarily conserved monooxygenase required for the biosynthesis of coenzyme Q10 (or ubiquinone), which is an essential component of the mitochondrial electron transport chain, and one of the most potent lipophilic antioxidants implicated in the protection of cell damage by reactive oxygen species. knockdown of this gene in mouse and zebrafish results in decreased growth due to increased apoptosis. Mutations in this gene are associated with autosomal recessive coenzyme Q10 deficiency-6

neg: VPS25 VPS25 Vacuolar protein-sorting-associated protein 25 is a protein that in humans is encoded by the "VPS25" gene. It is a component of the endosome-associated complex ESCRT-II (Endosomal Sorting Complexes Required for Transport protein II). ESCRT (ESCRT-I, -II, -III) complexes orchestrate efficient sorting of ubiquitinated transmembrane receptors to lysosomes via multivesicular bodies (MVBs). ESCRT-II recruits the transport machinery for protein sorting at MVB. In addition, the human ESCRT-II has been shown to form a complex with RNA polymerase II elongation factor ELL in order to exert transcriptional control activity. ESCRT-II transiently associates with the endosomal membrane and thereby initiates the formation

neg: Oxidative phosphorylation removed from NADH and passed to oxygen through a series of enzymes that each release a small amount of the energy. This set of enzymes, consisting of complexes I through IV, is called the electron transport chain and is found in the inner membrane of the mitochondrion. Succinate is also oxidized by the electron transport chain, but feeds into the pathway at a different point. In eukaryotes, the enzymes in this electron transport system use the energy released from the oxidation of NADH to pump protons across the inner membrane of the mitochondrion. This causes protons to build up in

neg: ProtCID ProtCID The Protein Common Interface Database (ProtCID) is a database of similar protein-protein interfaces in crystal structures of homologous proteins. Its main goal is to identify and cluster homodimeric and heterodimeric interfaces observed in multiple crystal forms of homologous proteins. Such interfaces, especially of non-identical proteins or protein complexes, have been associated with biologically relevant interactions. A common interface in ProtCID indicates chain-chain interactions that occur in different crystal forms. All protein sequences of known structure in the Protein Data Bank (PDB) are assigned a ”Pfam chain architecture”, which denotes the ordered Pfam assignments for that sequence, e.g. (Pkinase) or

neg: NDH-2 NDH-2 NDH-2, also known as type II NADH:quinone oxidoreductase or alternative NADH dehydrogenase, it's an enzyme (EC: 1.6.99.3) which catalyzes the electron transfer from NADH (electron donor) to a quinone (electron acceptor), being part of the electron transport chain. NDH-2 are peripheral membrane protein, functioning as dimers "in vivo", with approximately 45 KDa per subunit and a single FAD as their cofactor. NDH-2 are the only enzymes, with NADH dehydrogenase activity, expressed in the respiratory chain of some pathogenic organisms (e.g. "Staphylococcus aureus"), and for that they have been proposed as new targets for rational drug design. The structure/fold from

neg: Ecto-nox disulfide-thiol exchanger 1 Ecto-nox disulfide-thiol exchanger 1 Ecto-NOX disulfide-thiol exchanger 1 is a protein that in humans is encoded by the ENOX1 gene. Electron transport pathways are generally associated with mitochondrial membranes, but non-mitochondrial pathways are also biologically significant. Plasma membrane electron transport pathways are involved in functions as diverse as cellular defense, intracellular redox homeostasis, and control of cell growth and survival. Members of the ecto-NOX family, such as CNOX, or ENOX1, are involved in plasma membrane transport pathways. These enzymes exhibit both a hydroquinone (NADH) oxidase activity and a protein disulfide-thiol interchange activity in series, with each activity cycling every 22

neg: Polyacetylene of the creation of charge-transfer complexes between the polymer and halogen. Charge-transfer occurs from the polymer to the acceptor compound; the polyacetylene chain acts as a cation and the acceptor as an anion. The “hole” on the polymer backbone is weakly associated with the anionic acceptor by Coulomb potential. Polyacetylene doped with (p-type) dopants retain their high conductivity even after exposure to air for several days. Electron-donating (n-type) dopants can also be used to create conductive polyacetylene. n-Type dopants for polyacetylene include lithium, sodium, and potassium. As with p-type dopants, charge-transfer complexes are created, where the polymer backbone is anionic

neg: Ecto-nox disulfide-thiol exchanger 1 to 26 minutes (Scarlett et al., 2005 [PubMed 15882838]). Ecto-nox disulfide-thiol exchanger 1 Ecto-NOX disulfide-thiol exchanger 1 is a protein that in humans is encoded by the ENOX1 gene. Electron transport pathways are generally associated with mitochondrial membranes, but non-mitochondrial pathways are also biologically significant. Plasma membrane electron transport pathways are involved in functions as diverse as cellular defense, intracellular redox homeostasis, and control of cell growth and survival. Members of the ecto-NOX family, such as CNOX, or ENOX1, are involved in plasma membrane transport pathways. These enzymes exhibit both a hydroquinone (NADH) oxidase activity and a protein disulfide-thiol interchange