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AASDH | aminoadipate-semialdehyde dehydrogenase (1098 aa) | |||
TOMM22 | translocase of outer mitochondrial membrane 22 homolog (yeast); Central receptor component of the translocase of the outer membrane of mitochondria (TOM complex) responsible for the recognition and translocation of cytosolically synthesized mitochondrial preproteins. Together with the peripheral receptor TOM20 functions as the transit peptide receptor and facilitates the movement of preproteins into the translocation pore (142 aa) | |||
HSPA8 | heat shock 70kDa protein 8; Acts as a repressor of transcriptional activation. Inhibits the transcriptional coactivator activity of CITED1 on Smad-mediated transcription. Chaperone. Component of the PRP19- CDC5L complex that forms an integral part of the spliceosome and is required for activating pre-mRNA splicing. May have a scaffolding role in the spliceosome assembly as it contacts all other components of the core complex (646 aa) | |||
MTX2 | metaxin 2; Involved in transport of proteins into the mitochondrion (263 aa) | |||
TOMM40 | translocase of outer mitochondrial membrane 40 homolog (yeast); Channel-forming protein essential for import of protein precursors into mitochondria (By similarity) (361 aa) | |||
SQRDL | sulfide quinone reductase-like (yeast); Catalyzes the oxidation of hydrogen sulfide, with the help of a quinone (By similarity) (450 aa) | |||
CHCHD3 | coiled-coil-helix-coiled-coil-helix domain containing 3; Required for maintenance of mitochondrial crista integrity and mitochondrial function. May act as a scaffolding protein that stabilizes protein complexes involved in crista architecture and protein import. Has also been shown to function as a transcription factor which binds to the BAG1 promoter and represses BAG1 transcription (227 aa) | |||
NDUFS3 | NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase); 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) (264 aa) | |||
SDHA | succinate dehydrogenase complex, subunit A, flavoprotein (Fp); Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Can act as a tumor suppressor (664 aa) | |||
TOMM70A | translocase of outer mitochondrial membrane 70 homolog A (S. cerevisiae); Receptor that accelerates the import of all mitochondrial precursor proteins (By similarity) (608 aa) | |||
CHCHD6 | coiled-coil-helix-coiled-coil-helix domain containing 6; Required for maintaining mitochondrial crista morphology, ATP production and oxygen consumption (235 aa) | |||
NDUFB8 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 8, 19kDa; 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 (186 aa) | |||
MRPL11 | mitochondrial ribosomal protein L11 (192 aa) | |||
NDUFS8 | NADH dehydrogenase (ubiquinone) Fe-S protein 8, 23kDa (NADH-coenzyme Q reductase); 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). May donate electrons to ubiquinone (210 aa) | |||
CYC1 | cytochrome c-1; This is the heme-containing component of the cytochrome b-c1 complex, which accepts electrons from Rieske protein and transfers electrons to cytochrome c in the mitochondrial respiratory chain (325 aa) | |||
MRPS11 | mitochondrial ribosomal protein S11 (194 aa) | |||
UBC | ubiquitin C (685 aa) | |||
SAMM50 | sorting and assembly machinery component 50 homolog (S. cerevisiae); May be required for the assembly pathway of mitochondrial outer membrane proteins (By similarity) (469 aa) | |||
TOMM40L | translocase of outer mitochondrial membrane 40 homolog (yeast)-like; Potential channel-forming protein implicated in import of protein precursors into mitochondria (By similarity) (308 aa) | |||
MTX1 | metaxin 1; Involved in transport of proteins into the mitochondrion. Essential for embryonic development (By similarity) (466 aa) | |||
GLDC | glycine dehydrogenase (decarboxylating); The glycine cleavage system catalyzes the degradation of glycine. The P protein binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO(2) is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein (1020 aa) | |||
TOMM6 | translocase of outer mitochondrial membrane 6 homolog (yeast) (74 aa) | |||
IMMT | inner membrane protein, mitochondrial (758 aa) | |||
SLC25A17 | solute carrier family 25 (mitochondrial carrier; peroxisomal membrane protein, 34kDa), member 17; Peroxisomal transporter for multiple cofactors like coenzyme A (CoA), flavin adenine dinucleotide (FAD), flavin mononucleotide (FMN) and nucleotide adenosine monophosphate (AMP), and to a lesser extend for nicotinamide adenine dinucleotide (NAD(+)), adenosine diphosphate (ADP) and adenosine 3’,5’- diphosphate (PAP). May catalyze the transport of free CoA, FAD and NAD(+) from the cytosol into the peroxisomal matrix by a counter- exchange mechanism. Inhibited by pyridoxal 5’-phosphate and ba [...] (307 aa) | |||
MTX3 | metaxin 3; Could function in transport of proteins into the mitochondrion (By similarity) (251 aa) | |||
TOMM5 | translocase of outer mitochondrial membrane 5 homolog (yeast) (92 aa) |