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Details on Person UniProt:Q13057 COASY
| Class:Id | ReferenceGeneProduct:59947 |
|---|---|
| _chainChangeLog | chain:1-564 added on Sat February 7 2015 |
| _displayName | UniProt:Q13057 COASY |
| _timestamp | 2026-02-20 22:53:20 |
| chain | chain:1-564 |
| checksum | 7DC9E93B356C5DB7 |
| comment | FUNCTION Bifunctional enzyme that catalyzes the fourth step of the coenzyme A biosynthetic pathway, the adenylation of 4'-phosphopantetheine, and the fifth step, the phosphorylation of dephospho-CoA to CoA.CATALYTIC ACTIVITY (R)-4'-phosphopantetheine + ATP + H(+) = 3'-dephospho-CoA + diphosphateCATALYTIC ACTIVITY 3'-dephospho-CoA + ATP = ADP + CoA + H(+)BIOPHYSICOCHEMICAL PROPERTIES Cofactor biosynthesis; coenzyme A biosynthesis; CoA from (R)-pantothenate: step 4/5.PATHWAY Cofactor biosynthesis; coenzyme A biosynthesis; CoA from (R)-pantothenate: step 5/5.SUBUNIT Monomer.INTERACTION The protein is mainly cytosolic and active in the cytosol (PubMed:40925986). Present in the mitochondrial matrix, probably anchored to the inner mitochondrial membrane (PubMed:24360804).ALTERNATIVE PRODUCTS Expressed in all tissues examined including brain, heart, skeletal muscle, colon, thymus, spleen, kidney, liver, small intestine, placenta, lung and peripheral blood leukocyte. Lowest expression in peripheral blood leukocytes and highest in kidney and liver. Isoform 2 is expressed mainly in the brain.DOMAIN The phosphopantetheine adenylyltransferase domain catalyzes the fourth step of the coenzyme A biosynthetic pathway.DOMAIN The DPCK domain catalyzes the fifth step of the coenzyme A biosynthetic pathway.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.MISCELLANEOUS Major isoform which is active in the cytosol.MISCELLANEOUS The physiological significance of this isoform is uncertain.SIMILARITY In the central section; belongs to the eukaryotic CoaD family.SEQUENCE CAUTION Truncated N-terminus.SEQUENCE CAUTION Truncated N-terminus.SEQUENCE CAUTION Truncated N-terminus. |
| description | recommendedName: fullName evidence="15"Bifunctional coenzyme A synthase shortName: CoA synthase alternativeName: NBP alternativeName: POV-2 domain recommendedName: fullName evidence="15"Phosphopantetheine adenylyltransferase ecNumber evidence="3 7"2.7.7.3 alternativeName: Dephospho-CoA pyrophosphorylase alternativeName: Pantetheine-phosphate adenylyltransferase shortName: PPAT /domain domain recommendedName: fullName evidence="15"Dephospho-CoA kinase shortName: DPCK ecNumber evidence="3 7"2.7.1.24 alternativeName: Dephosphocoenzyme A kinase shortName: DPCOAK /domain |
| geneName | COASY PSEC0106 |
| identifier | Q13057 |
| isSequenceChanged | FALSE |
| keyword | Alternative splicing ATP-binding Coenzyme A biosynthesis Cytoplasm Disease variant Kinase Mitochondrion Multifunctional enzyme Neurodegeneration Nucleotide-binding Nucleotidyltransferase Phosphoprotein Proteomics identification Reference proteome Transferase |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9983091] Weiser, Joel, 2026-02-20 |
| name | COASY |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:9004045] ENSEMBL:ENSG00000068120 COASY [Homo sapiens] |
| secondaryIdentifier | COASY_HUMAN B2RA78 B4DLU0 Q6GS23 Q8NBM7 Q8NEW1 Q8WXD4 Q9NRM3 |
| sequenceLength | 564 |
| species | [Species:48887] Homo sapiens |
| (isoformParent) | [ReferenceIsoform:396118] UniProt:Q13057-2 COASY [Homo sapiens] [ReferenceIsoform:405556] UniProt:Q13057-1 COASY [Homo sapiens] |
| (referenceEntity) | [EntityWithAccessionedSequence:196735] COASY [mitochondrial outer membrane] [Homo sapiens] [EntityWithAccessionedSequence:9838038] COASY [mitochondrial matrix] [Homo sapiens] |
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No pathways have been reviewed or authored by UniProt:Q13057 COASY (59947)
