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Details on Person UniProt:Q13057 COASY

Class:IdReferenceGeneProduct:59947
_chainChangeLogchain:1-564 added on Sat February 7 2015
_displayNameUniProt:Q13057 COASY
_timestamp2026-02-20 22:53:20
chainchain:1-564
checksum7DC9E93B356C5DB7
commentFUNCTION 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.
descriptionrecommendedName: 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
geneNameCOASY
PSEC0106
identifierQ13057
isSequenceChangedFALSE
keywordAlternative 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
nameCOASY
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:9004045] ENSEMBL:ENSG00000068120 COASY [Homo sapiens]
secondaryIdentifierCOASY_HUMAN
B2RA78
B4DLU0
Q6GS23
Q8NBM7
Q8NEW1
Q8WXD4
Q9NRM3
sequenceLength564
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)