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Bifunctional 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.
Gene References into Functions
The recruitment of COASY inhibits CBP-mediated TPX2 acetylation, promoting TPX2 degradation for mitotic exit. PMID: 29531224
The COASY protein contains a mitochondrial localization signal, a regulatory region and a domain for each of the two catalytic kinase domains: adenyl transferase and dephospho CoA PMID: 27021474
DNA methylation levels in the COASY and SPINT1 promoter regions were considered to potentially be a convenient and useful biomarker for diagnosis of Alzheimer's Disease and Amnestic Mild Cognitive Impairment. PMID: 27992572
Exome sequencing revealed the presence of recessive missense mutations in COASY, encoding coenzyme A (CoA) synthase in neurodegeneration with brain iron accumulation PMID: 24360804
EDC4 might contribute to regulation of CoA biosynthesis in addition to its scaffold function in processing bodies PMID: 22982864
Identification and characterization of the gene encoding the human phosphopantetheine adenylyltransferase and dephospho-CoA kinase bifunctional enzyme (CoA synthase). PMID: 11994049
description of the existence of a novel CoA synthase isoform, which is the product of alternative splicing and possesses a 29aa extension at the N-terminus; termed it CoASy beta PMID: 16460672
CoA synthase is involved in signaling events in the cell and forms a functional complex with p85alphaPI3K in vivo. PMID: 19482007
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Involvement in disease
Neurodegeneration with brain iron accumulation 6 (NBIA6)
Subcellular Location
Cytoplasm. Mitochondrion matrix.
Protein Families
Eukaryotic CoaD family
Tissue Specificity
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 liv