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| COX6B1 | Cytochrome c oxidase subunit 6B1; Connects the two COX monomers into the physiological dimeric form; Mitochondrial complex IV- cytochrome c oxidase subunits (86 aa) | |||
| RAF1 | RAF proto-oncogene serine/threonine-protein kinase; Serine/threonine-protein kinase that acts as a regulatory link between the membrane-associated Ras GTPases and the MAPK/ERK cascade, and this critical regulatory link functions as a switch determining cell fate decisions including proliferation, differentiation, apoptosis, survival and oncogenic transformation. RAF1 activation initiates a mitogen-activated protein kinase (MAPK) cascade that comprises a sequential phosphorylation of the dual-specific MAPK kinases (MAP2K1/MEK1 and MAP2K2/MEK2) and the extracellular signal-regulated kina [...] (648 aa) | |||
| COQ9 | Ubiquinone biosynthesis protein COQ9, mitochondrial; Lipid-binding protein involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration. Binds a phospholipid of at least 10 carbons in each acyl group. May be required to present its bound-lipid to COQ7 (318 aa) | |||
| RAB2A | Ras-related protein Rab-2A; Required for protein transport from the endoplasmic reticulum to the Golgi complex; RAB, member RAS oncogene GTPases (212 aa) | |||
| MTIF2 | Translation initiation factor IF-2, mitochondrial; One of the essential components for the initiation of protein synthesis. Protects formylmethionyl-tRNA from spontaneous hydrolysis and promotes its binding to the 30S ribosomal subunits. Also involved in the hydrolysis of GTP during the formation of the 70S ribosomal complex; Belongs to the TRAFAC class translation factor GTPase superfamily. Classic translation factor GTPase family. IF-2 subfamily (727 aa) | |||
| ECSIT | Evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial; Adapter protein of the Toll-like and IL-1 receptor signaling pathway that is involved in the activation of NF-kappa-B via MAP3K1. Promotes proteolytic activation of MAP3K1. Involved in the BMP signaling pathway. Required for normal embryonic development (By similarity); Belongs to the ECSIT family (431 aa) | |||
| TMEM70 | Transmembrane protein 70, mitochondrial; Involved in biogenesis of mitochondrial ATP synthase; Belongs to the TMEM70 family (260 aa) | |||
| ACSS1 | Acetyl-coenzyme A synthetase 2-like, mitochondrial; Important for maintaining normal body temperature during fasting and for energy homeostasis. Essential for energy expenditure under ketogenic conditions (By similarity). Converts acetate to acetyl-CoA so that it can be used for oxidation through the tricarboxylic cycle to produce ATP and CO(2); Belongs to the ATP-dependent AMP-binding enzyme family (689 aa) | |||
| CHDH | Choline dehydrogenase, mitochondrial; Choline dehydrogenase (594 aa) | |||
| COQ6 | Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial; FAD-dependent monooxygenase required for the C5-ring hydroxylation during ubiquinone biosynthesis. Catalyzes the hydroxylation of 3-polyprenyl-4-hydroxybenzoic acid to 3- polyprenyl-4,5-dihydroxybenzoic acid. The electrons required for the hydroxylation reaction may be funneled indirectly from NADPH via a ferredoxin/ferredoxin reductase system to COQ6 (468 aa) | |||
| RTN4 | Reticulon-4; Developmental neurite growth regulatory factor with a role as a negative regulator of axon-axon adhesion and growth, and as a facilitator of neurite branching. Regulates neurite fasciculation, branching and extension in the developing nervous system. Involved in down-regulation of growth, stabilization of wiring and restriction of plasticity in the adult CNS. Regulates the radial migration of cortical neurons via an RTN4R-LINGO1 containing receptor complex (By similarity). Isoform 2 reduces the anti-apoptotic activity of Bcl-xl and Bcl-2. This is likely consecutive to thei [...] (1192 aa) | |||
| NDUFA4 | Cytochrome c oxidase subunit NDUFA4; Cytochrome c oxidase (COX, complex IV) is the terminal component of the mitochondrial respiratory chain that catalyzes the reduction of oxygen to water. Required for complex IV maintenance (81 aa) | |||
| C15orf48 | Normal mucosa of esophagus-specific gene 1 protein; Chromosome 15 open reading frame 48; Belongs to the complex I NDUFA4 subunit family (83 aa) | |||
| IARS2 | Isoleucine--tRNA ligase, mitochondrial; isoleucyl-tRNA synthetase 2, mitochondrial; Aminoacyl tRNA synthetases, Class I (1012 aa) | |||
| NDUFAF4 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4; Involved in the assembly of mitochondrial NADH-ubiquinone oxidoreductase complex (complex I). May be involved in cell proliferation and survival of hormone-dependent tumor cells. May be a regulator of breast tumor cell invasion (175 aa) | |||
| CPT2 | Carnitine O-palmitoyltransferase 2, mitochondrial; Carnitine palmitoyltransferase 2 (658 aa) | |||
| NDUFS5 | NADH dehydrogenase [ubiquinone] iron-sulfur protein 5; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (106 aa) | |||
| HSPD1 | 60 kDa heat shock protein, mitochondrial; Chaperonin implicated in mitochondrial protein import and macromolecular assembly. Together with Hsp10, facilitates the correct folding of imported proteins. May also prevent misfolding and promote the refolding and proper assembly of unfolded polypeptides generated under stress conditions in the mitochondrial matrix. The functional units of these chaperonins consist of heptameric rings of the large subunit Hsp60, which function as a back-to-back double ring. In a cyclic reaction, Hsp60 ring complexes bind one unfolded substrate protein per rin [...] (573 aa) | |||
| C17orf89 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8; Involved in the assembly of mitochondrial NADH-ubiquinone oxidoreductase complex (complex I, MT-ND1). Required to stabilize NDUFAF5 (74 aa) | |||
| CPS1 | Carbamoyl-phosphate synthase [ammonia], mitochondrial; Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell (1506 aa) | |||
| RPS20 | Ribosomal protein S20 (142 aa) | |||
| ACSF3 | Acyl-CoA synthetase family member 3, mitochondrial; Catalyzes the initial reaction in intramitochondrial fatty acid synthesis, by activating malonate and methylmalonate, but not acetate, into their respective CoA thioester. May have some preference toward very-long-chain substrates; Acyl-CoA synthetase family (576 aa) | |||
