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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) | |||
DNAJB1 | DnaJ (Hsp40) homolog, subfamily B, member 1; Interacts with HSP70 and can stimulate its ATPase activity. Stimulates the association between HSC70 and HIP (340 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) | |||
COX6A2 | cytochrome c oxidase subunit VIa polypeptide 2; This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (97 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) | |||
TMEM70 | transmembrane protein 70; Involved in biogenesis of mitochondrial ATP synthase (260 aa) | |||
SFXN1 | sideroflexin 1; Might be involved in the transport of a component required for iron utilization into or out of the mitochondria (322 aa) | |||
COX5A | cytochrome c oxidase subunit Va; This is the heme A-containing chain of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (150 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) | |||
SFXN4 | sideroflexin 4; Potential iron transporter (By similarity) (337 aa) | |||
MT-CO1 | mitochondrially encoded cytochrome c oxidase I; Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1- 3 form the functional core of the enzyme complex. CO I is the catalytic subunit of the enzyme. Electrons originating in cytochrome c are transferred via the copper A center of subunit 2 and heme A of subunit 1 to the bimetallic center formed by heme A3 and copper B (By similarity) (513 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) |