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GCDH | glutaryl-CoA dehydrogenase; Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive (438 aa) | |||
EHHADH | enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase (723 aa) | |||
ACADS | acyl-CoA dehydrogenase, C-2 to C-3 short chain (412 aa) | |||
ACAT1 | acetyl-CoA acetyltransferase 1; Plays a major role in ketone body metabolism (427 aa) | |||
FADS2 | fatty acid desaturase 2; Component of a lipid metabolic pathway that catalyzes biosynthesis of highly unsaturated fatty acids (HUFA) from precursor essential polyunsaturated fatty acids (PUFA) linoleic acid (LA) (18-2n-6) and alpha-linolenic acid (ALA) (18-3n-3). Catalyzes the first and rate limiting step in this pathway which is the desaturation of LA (18-2n-6) and ALA (18-3n-3) into gamma- linoleic acid (GLA) (18-3n-6) and stearidonic acid (18-4n-3) respectively and other desaturation steps. Highly unsaturated fatty acids (HUFA) play pivotal roles in many biological functions. It cat [...] (444 aa) | |||
ACAD8 | acyl-CoA dehydrogenase family, member 8; Has very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Plays a role in transcriptional coactivation within the ARC complex (415 aa) | |||
ACAA2 | acetyl-CoA acyltransferase 2; Abolishes BNIP3-mediated apoptosis and mitochondrial damage (397 aa) | |||
OTUD6B | OTU domain containing 6B (323 aa) | |||
ACOX1 | acyl-CoA oxidase 1, palmitoyl (660 aa) | |||
PRODH2 | proline dehydrogenase (oxidase) 2; Converts proline to delta-1-pyrroline-5-carboxylate (Probable) (536 aa) | |||
ACAD9 | acyl-CoA dehydrogenase family, member 9; Has a dehydrogenase activity on palmitoyl-CoA (C16-0) and stearoyl-CoA (C18-0). It is three times more active on palmitoyl-CoA than on stearoyl-CoA. Has little activity on octanoyl-CoA (C8-0), butyryl-CoA (C4-0) or isovaleryl-CoA (5-0) (621 aa) | |||
YOD1 | YOD1 OTU deubiquinating enzyme 1 homolog (S. cerevisiae); Hydrolase that can remove conjugated ubiquitin from proteins and participates in endoplasmic reticulum-associated degradation (ERAD) for misfolded lumenal proteins. May act by triming the ubiquitin chain on the associated substrate to facilitate their threading through the VCP/p97 pore. Ubiquitin moieties on substrates may present a steric impediment to the threading process when the substrate is transferred to the VCP pore and threaded through VCP’s axial channel. Mediates deubiquitination of both ’Lys-48’- and ’Lys-63’-linked [...] (348 aa) | |||
ACSM5 | acyl-CoA synthetase medium-chain family member 5; Has medium-chain fatty acid-CoA ligase activity with broad substrate specificity (in vitro). Acts on acids from C(4) to C(11) and on the corresponding 3-hydroxy- and 2,3- or 3,4- unsaturated acids (in vitro) (By similarity) (579 aa) | |||
ACAA1 | acetyl-CoA acyltransferase 1 (424 aa) | |||
OTUD6A | OTU domain containing 6A (288 aa) | |||
ACADVL | acyl-CoA dehydrogenase, very long chain; Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons (655 aa) | |||
PRODH | proline dehydrogenase (oxidase) 1 (600 aa) | |||
ACAT2 | acetyl-CoA acetyltransferase 2 (397 aa) | |||
ACADSB | acyl-CoA dehydrogenase, short/branched chain; Has greatest activity toward short branched chain acyl- CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl- CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent (432 aa) | |||
SCP2 | sterol carrier protein 2; Mediates in vitro the transfer of all common phospholipids, cholesterol and gangliosides between membranes. May play a role in regulating steroidogenesis (547 aa) | |||
HADHA | hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit; Bifunctional subunit (763 aa) | |||
TRPT1 | tRNA phosphotransferase 1; Catalyzes the last step of tRNA splicing, the transfer of the splice junction 2’-phosphate from ligated tRNA to NAD to produce ADP-ribose 1’’-2’’ cyclic phosphate (Probable) (255 aa) | |||
ACADM | acyl-CoA dehydrogenase, C-4 to C-12 straight chain; This enzyme is specific for acyl chain lengths of 4 to 16 (425 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) | |||
ALDH6A1 | aldehyde dehydrogenase 6 family, member A1; Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA (535 aa) |