Q13057.4
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
Bifunctional coenzyme A synthase
NCBI Official Synonym Full Names
Coenzyme A synthase
NCBI Official Synonym Symbols
NBP; DPCK; PPAT; UKR1; NBIA6; pOV-2 [Similar Products]
NCBI Protein Information
bifunctional coenzyme A synthase
UniProt Protein Name
Bifunctional coenzyme A synthase
UniProt Synonym Protein Names
NBP; POV-2Including the following 2 domains:Phosphopantetheine adenylyltransferase (EC:2.7.7.3)Alternative name(s):Dephospho-CoA pyrophosphorylase; Pantetheine-phosphate adenylyltransferase; PPAT
UniProt Synonym Gene Names
NCBI Summary for COASY
Coenzyme A (CoA) functions as a carrier of acetyl and acyl groups in cells and thus plays an important role in numerous synthetic and degradative metabolic pathways in all organisms. In eukaryotes, CoA and its derivatives are also involved in membrane trafficking and signal transduction. This gene encodes the bifunctional protein coenzyme A synthase (CoAsy) which carries out the last two steps in the biosynthesis of CoA from pantothenic acid (vitamin B5). The phosphopantetheine adenylyltransferase domain of this bifunctional protein catalyzes the conversion of 4'-phosphopantetheine into dephospho-coenzyme A (dpCoA) while its dephospho-CoA kinase domain completes the final step by phosphorylating dpCoA to form CoA. Mutations in this gene are associated with neurodegeneration with brain iron accumulation (NBIA). Alternative splicing results in multiple isoforms. [provided by RefSeq, Apr 2014]
UniProt Comments for COASY
PPAT: bifunctional cytoplasmic enzyme that catalyzes the fourth and fifth sequential steps of CoA biosynthetic pathway. The fourth reaction is catalyzed by the phosphopantetheine adenylyltransferase, coded by the coaD domain; the fifth reaction is catalyzed by the dephospho-CoA kinase, coded by the coaE domain. May act as a point of CoA biosynthesis regulation.
Protein type: Cofactor and Vitamin Metabolism - pantothenate and CoA biosynthesis; EC 2.7.1.24; EC 2.7.7.3; Kinase, other; Transferase
Chromosomal Location of Human Ortholog: 17q21.2
Cellular Component: mitochondrial matrix; mitochondrial outer membrane
Molecular Function: dephospho-CoA kinase activity; pantetheine-phosphate adenylyltransferase activity; protein binding
Biological Process: coenzyme A biosynthetic process; coenzyme biosynthetic process
Disease: Neurodegeneration With Brain Iron Accumulation 6
Research Articles on COASY
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Pathways associated with anti-COASY antibody
Diseases associated with anti-COASY antibody
Organs/Tissues associated with anti-COASY antibody
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