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NDUFAB1 | NADH dehydrogenase (ubiquinone) 1, alpha/beta subcomplex, 1, 8kDa; Carrier of the growing fatty acid chain in fatty acid biosynthesis in mitochondria. Accessory and non-catalytic subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), which functions in the transfer of electrons from NADH to the respiratory chain (By similarity) (156 aa) | |||
ATP5E | ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit; 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 F(1) is coupled [...] (51 aa) | |||
KIAA0368 | KIAA0368; Adapter/scaffolding protein that binds to the 26S proteasome, motor proteins and other compartment specific proteins. May couple the proteasome to different compartments including endosome, endoplasmic reticulum and centrosome. May play a role in ERAD and other enhanced proteolyis (2017 aa) | |||
ELAC1 | elaC homolog 1 (E. coli); Zinc phosphodiesterase, which displays some tRNA 3’- processing endonuclease activity. Probably involved in tRNA maturation, by removing a 3’-trailer from precursor tRNA (363 aa) | |||
OXSM | 3-oxoacyl-ACP synthase, mitochondrial; May play a role in the biosynthesis of lipoic acid as well as longer chain fatty acids required for optimal mitochondrial function (459 aa) | |||
EXOG | endo/exonuclease (5’-3’), endonuclease G-like; Endo/exonuclease with nicking activity towards supercoiled DNA, a preference for single stranded DNA and 5’-3’ exonuclease activity (368 aa) | |||
MCAT | malonyl CoA-ACP acyltransferase (mitochondrial); Catalyzes the transfer of a malonyl moiety from malonyl- CoA to the free thiol group of the phosphopantetheine arm of the mitochondrial ACP protein (NDUFAB1). This suggests the existence of the biosynthesis of fatty acids in mitochondrias (390 aa) | |||
ATP5J2 | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit F2; 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 F(1) is coupled via a [...] (94 aa) | |||
ATP5H | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit d; 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 F(1) is coupled via a [...] (161 aa) | |||
FASN | fatty acid synthase (2511 aa) | |||
UBB | ubiquitin B (229 aa) | |||
ELOVL6 | ELOVL fatty acid elongase 6; Condensing enzyme that catalyzes the synthesis of saturated and monounsaturated fatty acids. Highest activity toward C16-0 acyl-CoAs (265 aa) | |||
NCBP2 | nuclear cap binding protein subunit 2, 20kDa; Component of the cap-binding complex (CBC), which binds co-transcriptionally to the 5’ cap of pre-mRNAs and is involved in various processes such as pre-mRNA splicing, translation regulation, nonsense-mediated mRNA decay, RNA-mediated gene silencing (RNAi) by microRNAs (miRNAs) and mRNA export. The CBC complex is involved in mRNA export from the nucleus via its interaction with ALYREF/THOC4/ALY, leading to the recruitment of the mRNA export machinery to the 5’ end of mRNA and to mRNA export in a 5’ to 3’ direction through the nuclear pore. [...] (156 aa) | |||
ELAC2 | elaC homolog 2 (E. coli); Zinc phosphodiesterase, which displays some tRNA 3’- processing endonuclease activity. Probably involved in tRNA maturation, by removing a 3’-trailer from precursor tRNA (826 aa) | |||
ACACB | acetyl-CoA carboxylase beta; ACC-beta may be involved in the provision of malonyl-CoA or in the regulation of fatty acid oxidation, rather than fatty acid biosynthesis. Carries out three functions- biotin carboxyl carrier protein, biotin carboxylase and carboxyltransferase (2458 aa) | |||
ACACA | acetyl-CoA carboxylase alpha (2383 aa) | |||
NCBP2L | nuclear cap binding protein subunit 2-like (153 aa) | |||
ENDOG | endonuclease G; Cleaves DNA at double-stranded (DG)n.(DC)n and at single-stranded (DC)n tracts. In addition to deoxyribonuclease activities, also has ribonuclease (RNase) and RNase H activities. Capable of generating the RNA primers required by DNA polymerase gamma to initiate replication of mitochondrial DNA (By similarity) (297 aa) | |||
OLAH | oleoyl-ACP hydrolase; In fatty acid biosynthesis chain termination and release of the free fatty acid product is achieved by hydrolysis of the thio ester by a thioesterase I, a component of the fatty acid synthetase complex. The chain length of the released fatty acid is usually C16. However, in the mammary glands of non-ruminant mammals, and in the uropygial gland of certain waterfowl there exists a second thioesterase which releases medium-chain length fatty acids (C8 to C2) (By similarity) (318 aa) | |||
ATP5EP2 | ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit pseudogene 2; 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 F( [...] (51 aa) | |||
ATP5J | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit F6; 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 F(1) is coupled via a [...] (116 aa) | |||
ENSG00000248919 | ATP5J2-PTCD1 readthrough (749 aa) | |||
HSD17B4 | hydroxysteroid (17-beta) dehydrogenase 4; Bifunctional enzyme acting on the peroxisomal beta- oxidation pathway for fatty acids. Catalyzes the formation of 3- ketoacyl-CoA intermediates from both straight-chain and 2-methyl- branched-chain fatty acids (761 aa) | |||
ENSG00000255154 | Hydroxyacyl-thioester dehydratase type 2, mitochondrial ; Mitochondrial 3-hydroxyacyl-thioester dehydratase, which may be involved in fatty acid biosynthesis (168 aa) | |||
PTCD1 | pentatricopeptide repeat domain 1; Mitochondrial protein implicated in negative regulation of leucine tRNA levels, as well as negative regulation of mitochondria-encoded proteins and COX activity. Affects also the 3’ processing of mitochondrial tRNAs (749 aa) | |||
ENSG00000267699 | Uncharacterized protein (86 aa) |