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| GCDH | Glutaryl-CoA dehydrogenase, mitochondrial; 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; Belongs to the acyl-CoA dehydrogenase family (438 aa) | |||
| ACADL | Long-chain specific acyl-CoA dehydrogenase, mitochondrial; acyl-CoA dehydrogenase long chain; Belongs to the acyl-CoA dehydrogenase family (430 aa) | |||
| ACADS | Short-chain specific acyl-CoA dehydrogenase, mitochondrial; Introduces a double bond at position 2 in saturated acyl-CoA’s of short chain length, i.e. less than 6 carbon atoms; Belongs to the acyl-CoA dehydrogenase family (412 aa) | |||
| MECR | Enoyl-[acyl-carrier-protein] reductase, mitochondrial; Catalyzes the NADPH-dependent reduction of trans-2-enoyl thioesters in mitochondrial fatty acid synthesis (fatty acid synthesis type II). Fatty acid chain elongation in mitochondria uses acyl carrier protein (ACP) as an acyl group carrier, but the enzyme accepts both ACP and CoA thioesters as substrates in vitro. Has a preference for short and medium chain substrates, including trans-2-hexenoyl-CoA (C6), trans-2-decenoyl-CoA (C10), and trans- 2-hexadecenoyl-CoA (C16) (373 aa) | |||
| SEC22C | Vesicle-trafficking protein SEC22c; May be involved in vesicle transport between the ER and the Golgi complex; SNAREs (303 aa) | |||
| ACAT1 | Acetyl-CoA acetyltransferase, mitochondrial; Plays a major role in ketone body metabolism (427 aa) | |||
| ARHGAP26 | Rho GTPase-activating protein 26; GTPase-activating protein for RHOA and CDC42 (814 aa) | |||
| ACAA2 | 3-ketoacyl-CoA thiolase, mitochondrial; Abolishes BNIP3-mediated apoptosis and mitochondrial damage (397 aa) | |||
| ACSM3 | Acyl-coenzyme A synthetase ACSM3, mitochondrial; 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); Acyl-CoA synthetase family (586 aa) | |||
| ACOX1 | Peroxisomal acyl-coenzyme A oxidase 1; Catalyzes the desaturation of acyl-CoAs to 2-trans- enoyl-CoAs. Isoform 1 shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length. Isoform 2 is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs. Isoform 2 is twice as active as isoform 1 against 16-hydroxy- palmitoyl-CoA and is 25% more active against 1,16-hexadecanodioyl- CoA (660 aa) | |||
| ACSM1 | Acyl-coenzyme A synthetase ACSM1, mitochondrial; 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). Functions as GTP-dependent lipoate- activating enzyme that generates the substrate for lipoyltransferase (By similarity); Belongs to the ATP-dependent AMP-binding enzyme family (577 aa) | |||
| HADHB | hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta (474 aa) | |||
| ACSM2B | Acyl-coenzyme A synthetase ACSM2B, mitochondrial; 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); Acyl-CoA synthetase family (577 aa) | |||
| ACSM5 | Acyl-coenzyme A synthetase ACSM5, mitochondrial; 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); Belongs to the ATP-dependent AMP-binding enzyme family (579 aa) | |||
| ACAA1 | 3-ketoacyl-CoA thiolase, peroxisomal; acetyl-CoA acyltransferase 1 (424 aa) | |||
| ACSM6 | Acyl-coenzyme A synthetase ACSM6, mitochondrial; acyl-CoA synthetase medium chain family member 6 (480 aa) | |||
| ACOX3 | Peroxisomal acyl-coenzyme A oxidase 3; Oxidizes the CoA-esters of 2-methyl-branched fatty acids; Belongs to the acyl-CoA oxidase family (700 aa) | |||
| ZNF830 | Zinc finger protein 830; Acts as an important regulator of the cell cycle that participates in the maintenance of genome integrity. During cell cycle progression in embryonic fibroblast, prevents replication fork collapse, double-strand break formation and cell cycle checkpoint activation. Controls mitotic cell cycle progression and cell survival in rapidly proliferating intestinal epithelium and embryonic stem cells. During the embryo preimplantation, controls different aspects of M phase. During early oocyte growth, plays a role in oocyte survival by preventing chromosomal breaks for [...] (372 aa) | |||
| ACAT2 | Acetyl-CoA acetyltransferase, cytosolic; acetyl-CoA acetyltransferase 2 (397 aa) | |||
| ACADSB | Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial; 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) | |||
| DBT | Dihydrolipoamide branched chain transacylase E2; 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). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component (482 aa) | |||
| ACADM | Medium-chain specific acyl-CoA dehydrogenase, mitochondrial; Acyl-CoA dehydrogenase specific for acyl chain lengths of 4 to 16 that catalyzes the initial step of fatty acid beta- oxidation. Utilizes the electron transfer flavoprotein (ETF) as an electron acceptor to transfer electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase) (454 aa) | |||
| CLIC4 | Chloride intracellular channel protein 4; Can insert into membranes and form poorly selective ion channels that may also transport chloride ions. Channel activity depends on the pH. Membrane insertion seems to be redox-regulated and may occur only under oxydizing conditions. Promotes cell- surface expression of HRH3. Has alternate cellular functions like a potential role in angiogenesis or in maintaining apical- basolateral membrane polarity during mitosis and cytokinesis. Could also promote endothelial cell proliferation and regulate endothelial morphogenesis (tubulogenesis) (253 aa) | |||
| SEPHS1 | Selenide, water dikinase 1; Synthesizes selenophosphate from selenide and ATP (392 aa) | |||
| ACSM4 | Acyl-coenzyme A synthetase ACSM4, mitochondrial; 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); Belongs to the ATP-dependent AMP-binding enzyme family (580 aa) | |||
| ACSM2A | Acyl-coenzyme A synthetase ACSM2A, mitochondrial; 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); Acyl-CoA synthetase family (577 aa) | |||
