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ACOT8 | acyl-CoA thioesterase 8; Acyl-CoA thioesterases are a group of enzymes that catalyze the hydrolysis of acyl-CoAs to the free fatty acid and coenzyme A (CoASH), providing the potential to regulate intracellular levels of acyl-CoAs, free fatty acids and CoASH. May mediate Nef-induced down-regulation of CD4. Major thioesterase in peroxisomes. Competes with BAAT (Bile acid CoA- amino acid N- acyltransferase) for bile acid-CoA substrate (such as chenodeoxycholoyl-CoA). Shows a preference for medium-length fatty acyl-CoAs (By similarity). May be involved in the metabolic regulation of peroxi [...] (319 aa) | |||
EHHADH | enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase (723 aa) | |||
ACADL | acyl-CoA dehydrogenase, long chain (430 aa) | |||
ACADS | acyl-CoA dehydrogenase, C-2 to C-3 short chain (412 aa) | |||
SBDS | Shwachman-Bodian-Diamond syndrome; Required for the assembly of mature ribosomes and ribosome biogenesis. Together with EFTUD1, triggers the GTP- dependent release of EIF6 from 60S pre-ribosomes in the cytoplasm, thereby activating ribosomes for translation competence by allowing 80S ribosome assembly and facilitating EIF6 recycling to the nucleus, where it is required for 60S rRNA processing and nuclear export. Required for normal levels of protein synthesis. May play a role in cellular stress resistance. May play a role in cellular response to DNA damage. May play a role in cell prol [...] (250 aa) | |||
SUGP1 | SURP and G patch domain containing 1; Plays a role in pre-mRNA splicing (645 aa) | |||
ACSS2 | acyl-CoA synthetase short-chain family member 2; Activates acetate so that it can be used for lipid synthesis or for energy generation (714 aa) | |||
MRPL53 | mitochondrial ribosomal protein L53 (112 aa) | |||
ACAT1 | acetyl-CoA acetyltransferase 1; Plays a major role in ketone body metabolism (427 aa) | |||
ACOX1 | acyl-CoA oxidase 1, palmitoyl (660 aa) | |||
CRAT | carnitine O-acetyltransferase; Carnitine acetylase is specific for short chain fatty acids. Carnitine acetylase seems to affect the flux through the pyruvate dehydrogenase complex. It may be involved as well in the transport of acetyl-CoA into mitochondria (626 aa) | |||
PEX7 | peroxisomal biogenesis factor 7; Binds to the N-terminal PTS2-type peroxisomal targeting signal and plays an essential role in peroxisomal protein import (323 aa) | |||
ACAA1 | acetyl-CoA acyltransferase 1 (424 aa) | |||
ACOX3 | acyl-CoA oxidase 3, pristanoyl; Oxidizes the CoA-esters of 2-methyl-branched fatty acids (By similarity) (700 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) | |||
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) | |||
HMGCL | 3-hydroxymethyl-3-methylglutaryl-CoA lyase; Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism (325 aa) | |||
PCCA | propionyl CoA carboxylase, alpha polypeptide (728 aa) | |||
HADHA | hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit; Bifunctional subunit (763 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) | |||
SRPRB | signal recognition particle receptor, B subunit; Component of the SRP (signal recognition particle) receptor. Ensures, in conjunction with the signal recognition particle, the correct targeting of the nascent secretory proteins to the endoplasmic reticulum membrane system. Has GTPase activity. May mediate the membrane association of SRPR (By similarity) (271 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) | |||
VDAC3 | voltage-dependent anion channel 3; Forms a channel through the mitochondrial outer membrane that allows diffusion of small hydrophilic molecules (By similarity) (284 aa) | |||
ALDH6A1 | aldehyde dehydrogenase 6 family, member A1; Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA (535 aa) |