ECHDC1 | |||||||||||||||||||||||||||||||||||||||||||||||||||
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Identifiers | |||||||||||||||||||||||||||||||||||||||||||||||||||
Aliases | ECHDC1 , HEL-S-76, MMCD, dJ351K20.2, ethylmalonyl-CoA decarboxylase 1 | ||||||||||||||||||||||||||||||||||||||||||||||||||
External IDs | OMIM: 612136 MGI: 1277169 HomoloGene: 23106 GeneCards: ECHDC1 | ||||||||||||||||||||||||||||||||||||||||||||||||||
EC number | 7.2.4.3 | ||||||||||||||||||||||||||||||||||||||||||||||||||
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Ethylmalonyl-CoA decarboxylase | |||||||||
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Identifiers | |||||||||
EC no. | 4.1.1.94 | ||||||||
Databases | |||||||||
IntEnz | IntEnz view | ||||||||
BRENDA | BRENDA entry | ||||||||
ExPASy | NiceZyme view | ||||||||
KEGG | KEGG entry | ||||||||
MetaCyc | metabolic pathway | ||||||||
PRIAM | profile | ||||||||
PDB structures | RCSB PDB PDBe PDBsum | ||||||||
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Ethylmalonyl-CoA decarboxylase (EC 4.1.1.94) is an enzyme with systematic name (S)-ethylmalonyl-CoA carboxy-lyase (butanoyl-CoA-forming). [5] This enzyme catalyses the following chemical reaction
The vertebrate enzyme decarboxylates ethylmalonyl-CoA.
GDP-fucose protein O-fucosyltransferase 2 (POFUT2) is an enzyme responsible for adding fucose sugars in O linkage to serine or threonine residues in Thrombospondin repeats. The protein is an inverting glycosyltransferase, which means that the enzyme uses GDP-β-L-fucose as a donor substrate and transfers the fucose in O linkage to the protein producing fucose-α-O-serine/threonine.
Uroporphyrinogen III decarboxylase is an enzyme that in humans is encoded by the UROD gene.
Phosphomevalonate kinase is an enzyme in the mevalonate pathway that in humans is encoded by the PMVK gene.
Dimethylallyltranstransferase (DMATT), also known as farnesylpyrophosphate synthase (FPPS) or as farnesyldiphosphate synthase (FDPS), is an enzyme that in humans is encoded by the FDPS gene and catalyzes the transformation of dimethylallylpyrophosphate (DMAPP) and isopentenyl pyrophosphate (IPP) into farnesylpyrophosphate (FPP).
Guanine deaminase also known as cypin, guanase, guanine aminase, GAH, and guanine aminohydrolase is an aminohydrolase enzyme which converts guanine to xanthine. Cypin is a major cytosolic protein that interacts with PSD-95. It promotes localized microtubule assembly in neuronal dendrites.
Zinc finger protein 281 is a protein that in humans is encoded by the ZNF281 gene.
Antizyme inhibitor 2 (AzI2) also erroneously known as arginine decarboxylase (ADC) is a protein that in humans is encoded by the AZIN2 gene. In contrast to initial suggestions, Antizyme inhibitor 2 does not act as arginine decarboxylase (ADC) in mammalian cells
UDP-glucuronic acid decarboxylase 1 is an enzyme that in humans is encoded by the UXS1 gene.
Glutamate decarboxylase 2 is an enzyme that in humans is encoded by the GAD2 gene.
Malonyl CoA-acyl carrier protein transacylase, mitochondrial is an enzyme that in humans is encoded by the MCAT gene.
Ornithine decarboxylase antizyme 2 is an enzyme that in humans is encoded by the OAZ2 gene.
UDP-glucuronosyltransferase 1-8 is an enzyme that in humans is encoded by the UGT1A8 gene.
UDP glucuronosyltransferase 2 family, polypeptide A2, also known as UGT2A2, is an enzyme that in humans is encoded by the UGT2A2 gene.
DIS3-like exonuclease 1 is an enzyme that in humans is encoded by the DIS3L gene. Its protein product is an RNase enzyme homologous to the yeast protein Rrp44, and can be part of the exosome complex in the cytoplasm of eukaryotic cells.
Glutathione peroxidase 8 (GPx-8) is an enzyme that in humans is encoded by the GPX8 gene. GPx-8 is a member of the glutathione peroxidase family.
3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial) is an enzyme in humans that is encoded by the HMGCS2 gene.
COQ3, also known as ubiquinone biosynthesis O-methyltransferase, mitochondrial is an enzyme that in humans is encoded by the COQ3 gene.
Inositol monophosphatase 3 also known as inositol monophosphatase domain-containing protein 1 (IMPAD1) is an enzyme that in humans is encoded by the IMPAD1 gene.
Lysocardiolipin acyltransferase 1 is a protein that in humans is encoded by the LCLAT1 gene.
Glutamate decarboxylase like 1 (GADL1) is the enzyme responsible for decarboxylating aspartate (Asp) to β-alanine and cysteine sulfinic acid (CSA) to hypotaurine. GADL1 is a Pyridoxal 5’-phosphate (PLP)-dependent enzyme. By decarboxylating Asp to β-alanine, GADL1 consequently plays a role in the production of carnosine. Carnosine and taurine have multiple biological functions such as calcium regulation, pH buffering, metal chelation, and antioxidant effects. β-Alanine also plays a role as neurotransmitter or neuromodulator in the central nervous system (CNS) and olfactory bulbs.