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TIMM10 | translocase of inner mitochondrial membrane 10 homolog (yeast); Mitochondrial intermembrane chaperone that participates in the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane. May also be required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space (90 aa) | |||
MTIF2 | mitochondrial translational initiation factor 2; 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 (727 aa) | |||
BCKDHA | branched chain keto acid dehydrogenase E1, alpha polypeptide; The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components- branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3) (445 aa) | |||
DLAT | dihydrolipoamide S-acetyltransferase; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle (647 aa) | |||
ATP5A1 | ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle; Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core, and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of [...] (553 aa) | |||
PDHA2 | pyruvate dehydrogenase (lipoamide) alpha 2; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle (388 aa) | |||
N6AMT1 | N-6 adenine-specific DNA methyltransferase 1 (putative); Heterodimeric methyltransferase that catalyzes N5- methylation of ETF1 on ’Gln-185’, using S-adenosyl L-methionine as methyl donor. ETF1 needs to be complexed to ERF3 in its GTP-bound form to be efficiently methylated. May play a role in the modulation of arsenic-induced toxicity. May be involved in the conversion of monomethylarsonous acid (3+) into the less toxic dimethylarsonic acid (214 aa) | |||
LETM1 | leucine zipper-EF-hand containing transmembrane protein 1; Crucial for the maintenance of mitochondrial tubular networks and for the assembly of the supercomplexes of the respiratory chain. Required for the maintenance of the tubular shape and cristae organization (739 aa) | |||
SLC25A33 | solute carrier family 25 (pyrimidine nucleotide carrier), member 33; Probable mitochondrial transporter which participates in dendritic cell endocytosis. May associate with mitochondrial oxidative phosphorylation (321 aa) | |||
PDHB | pyruvate dehydrogenase (lipoamide) beta; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle (359 aa) | |||
SLC25A36 | solute carrier family 25 (pyrimidine nucleotide carrier ), member 36 (311 aa) | |||
UBC | ubiquitin C (685 aa) | |||
CHM | choroideremia (Rab escort protein 1); Binds unprenylated Rab proteins, presents it to the catalytic Rab GGTase dimer, and remains bound to it after the geranylgeranyl transfer reaction. The component A is thought to be regenerated by transferring its prenylated Rab back to the donor membrane. Also a pre-formed complex consisting of CHM and the Rab GGTase dimer (RGGT or component B) can bind to and prenylate Rab proteins; this alternative pathway is proposed to be the predominant pathway for Rab protein geranylgeranylation (653 aa) | |||
GTPBP3 | GTP binding protein 3 (mitochondrial) (524 aa) | |||
MT-CYB | mitochondrially encoded cytochrome b; Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis (By similarity) (380 aa) | |||
CHML | choroideremia-like (Rab escort protein 2); Binds unprenylated Rab proteins, presents it to the catalytic Rab GGTase dimer, and remains bound to it after the geranylgeranyl transfer reaction. The component A is thought to be regenerated by transferring its prenylated Rab back to the donor membrane. Less effective than REP-1 in supporting prenylation of Rab3 family (656 aa) | |||
MTHFD1L | methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1-like; May provide the missing metabolic reaction required to link the mitochondria and the cytoplasm in the mammalian model of one-carbon folate metabolism in embryonic an transformed cells complementing thus the enzymatic activities of MTHFD2 (By similarity) (978 aa) | |||
SLC25A28 | solute carrier family 25 (mitochondrial iron transporter), member 28; Mitochondrial iron transporter that mediates iron uptake. Probably required for heme synthesis of hemoproteins and Fe-S cluster assembly in non-erythroid cells. The iron delivered into the mitochondria, presumably as Fe(2+), is then probably delivered to ferrochelatase to catalyze Fe(2+) incorporation into protoprophyrin IX to make heme (By similarity) (364 aa) | |||
MRS2 | MRS2 magnesium homeostasis factor homolog (S. cerevisiae); Magnesium transporter that may mediate the influx of magnesium into the mitochondrial matrix (443 aa) | |||
PDHA1 | pyruvate dehydrogenase (lipoamide) alpha 1 (428 aa) | |||
GDI2 | GDP dissociation inhibitor 2; Regulates the GDP/GTP exchange reaction of most Rab proteins by inhibiting the dissociation of GDP from them, and the subsequent binding of GTP to them (445 aa) | |||
GDI1 | GDP dissociation inhibitor 1; Regulates the GDP/GTP exchange reaction of most Rab proteins by inhibiting the dissociation of GDP from them, and the subsequent binding of GTP to them (447 aa) | |||
ATPAF2 | ATP synthase mitochondrial F1 complex assembly factor 2; May play a role in the assembly of the F1 component of the mitochondrial ATP synthase (ATPase) (289 aa) | |||
LETM2 | leucine zipper-EF-hand containing transmembrane protein 2 (444 aa) | |||
SLC25A37 | solute carrier family 25 (mitochondrial iron transporter), member 37; Mitochondrial iron transporter that specifically mediates iron uptake in developing erythroid cells, thereby playing an essential role in heme biosynthesis. The iron delivered into the mitochondria, presumably as Fe(2+), is then probably delivered to ferrochelatase to catalyze Fe(2+) incorporation into protoprophyrin IX to make heme (By similarity) (338 aa) | |||
MTHFD1 | methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1, methenyltetrahydrofolate cyclohydrolase, formyltetrahydrofolate synthetase (935 aa) |