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COX15 | cytochrome c oxidase assembly homolog 15 (yeast); May be involved in the biosynthesis of heme A (410 aa) | |||
TIMM21 | translocase of inner mitochondrial membrane 21 homolog (yeast); Participates in the translocation of transit peptide- containing proteins across the mitochondrial inner membrane. Also required for assembly of mitochondrial respiratory chain complex I and complex IV as component of some MITRAC complex, a cytochrome c oxidase (COX) assembly intermediate complex. TIM21 probably shuttles between the presequence translocase and respiratory-chain assembly intermediates in a process that promotes incorporation of early nuclear-encoded subunits into these complexes (248 aa) | |||
COA1 | cytochrome c oxidase assembly factor 1 homolog (S. cerevisiae); Component of some MITRAC complex, a cytochrome c oxidase (COX) assembly intermediate complex that regulates COX assembly. MITRAC complexes regulate both translation of mitochondrial encoded components and assembly of nuclear-encoded components imported in mitochondrion. Required for assembly of mitochondrial respiratory chain complex I and complex IV (146 aa) | |||
SFXN3 | sideroflexin 3; Potential iron transporter (325 aa) | |||
SLC25A11 | solute carrier family 25 (mitochondrial carrier; oxoglutarate carrier), member 11; Catalyzes the transport of 2-oxoglutarate across the inner mitochondrial membrane in an electroneutral exchange for malate or other dicarboxylic acids, and plays an important role in several metabolic processes, including the malate-aspartate shuttle, the oxoglutarate/isocitrate shuttle, in gluconeogenesis from lactate, and in nitrogen metabolism (314 aa) | |||
SLC25A3 | solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 3; Transport of phosphate groups from the cytosol to the mitochondrial matrix. Phosphate is cotransported with H(+). May play a role regulation of the mitochondrial permeability transition pore (mPTP) (362 aa) | |||
COX4I1 | cytochrome c oxidase subunit IV isoform 1; This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (169 aa) | |||
TACO1 | translational activator of mitochondrially encoded cytochrome c oxidase I; Acts as a translational activator of mitochondrially- encoded cytochrome c oxidase 1 (297 aa) | |||
NDUFAF1 | NADH dehydrogenase (ubiquinone) complex I, assembly factor 1; Chaperone protein involved in the assembly of the mitochondrial NADH-ubiquinone oxidoreductase complex (complex I) (By similarity) (327 aa) | |||
ECSIT | ECSIT homolog (Drosophila); 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) (431 aa) | |||
TMEM177 | transmembrane protein 177 (311 aa) | |||
PNKD | paroxysmal nonkinesigenic dyskinesia; Probable hydrolase that plays an aggravative role in the development of cardiac hypertrophy via activation of the NF-kappa- B signaling pathway (By similarity) (385 aa) | |||
NDUFC2 | NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 2, 14.5kDa; 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 (By similarity) (119 aa) | |||
COX6C | cytochrome c oxidase subunit VIc; This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (75 aa) | |||
COX11 | cytochrome c oxidase assembly homolog 11 (yeast); Exerts its effect at some terminal stage of cytochrome c oxidase synthesis, probably by being involved in the insertion of the copper B into subunit I (By similarity) (276 aa) | |||
TMEM223 | transmembrane protein 223 (202 aa) | |||
COX14 | cytochrome c oxidase assembly homolog 14 (S. cerevisiae); Plays a role in the assembly or stability of the cytochrome c oxidase complex (COX). Requires for coordination of the early steps of COX assembly with the synthesis of MT-CO1 (57 aa) | |||
UBC | ubiquitin C (685 aa) | |||
COA3 | cytochrome c oxidase assembly factor 3; Component of some MITRAC complex, a cytochrome c oxidase (COX) assembly intermediate complex that regulates COX assembly. MITRAC complexes regulate both translation of mitochondrial encoded components and assembly of nuclear-encoded components imported in mitochondrion. Required for efficient translation of MT-CO1 and mitochondrial respiratory chain complex IV assembly (106 aa) | |||
MT-CO3 | mitochondrially encoded cytochrome c oxidase III; Subunits I, II and III form the functional core of the enzyme complex (By similarity) (261 aa) | |||
MT-ND2 | mitochondrially encoded NADH dehydrogenase 2; Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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 (By similarity) (347 aa) | |||
OMA1 | OMA1 zinc metallopeptidase homolog (S. cerevisiae); Metalloprotease that is part of the quality control system in the inner membrane of mitochondria. Following stress conditions that induce loss of mitochondrial membrane potential, mediates cleavage of OPA1 at S1 position, leading to OPA1 inactivation and negative regulation of mitochondrial fusion. Its role in mitochondrial quality control is essential for regulating lipid metabolism as well as to maintain body temperature and energy expenditure under cold-stress conditions (524 aa) | |||
SURF1 | surfeit 1; Probably involved in the biogenesis of the COX complex (300 aa) | |||
SLC25A12 | solute carrier family 25 (aspartate/glutamate carrier), member 12; Catalyzes the calcium-dependent exchange of cytoplasmic glutamate with mitochondrial aspartate across the mitochondrial inner membrane. May have a function in the urea cycle (678 aa) | |||
CMC1 | COX assembly mitochondrial protein 1 homolog (S. cerevisiae); Required for mitochondrial cytochrome c oxidase (COX) assembly and respiration (By similarity) (106 aa) | |||
TIMMDC1 | translocase of inner mitochondrial membrane domain containing 1 (285 aa) |