COASY

Bifunctional coenzyme A synthase is an enzyme that in mammals is encoded by the COASY gene that catalyses the synthesis of coenzyme A from 4'-phosphopantetheine.^[5][6][7]

COASY
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 2F6R
Identifiers
Aliases	COASY, DPCK, NBIA6, NBP, PPAT, UKR1, pOV-2, Coenzyme A synthase, PCH12
External IDs	OMIM: 609855; MGI: 1918993; HomoloGene: 11889; GeneCards: COASY; OMA:COASY - orthologs
Gene location (Human) Chr. Chromosome 17 (human)[1] Band 17q21.2 Start 42,561,467 bp[1] End 42,566,277 bp[1]
Gene location (Mouse) Chr. Chromosome 11 (mouse)[2] Band 11|11 D Start 100,973,391 bp[2] End 100,977,445 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in mucosa of transverse colon parotid gland body of pancreas right adrenal cortex right lobe of liver left adrenal gland left adrenal cortex right lobe of thyroid gland body of stomach left lobe of thyroid gland Top expressed in right kidney proximal tubule human kidney left lobe of liver brown adipose tissue external carotid artery internal carotid artery supraoptic nucleus lacrimal gland tunica adventitia of aorta More reference expression data BioGPS More reference expression data
Gene ontology Molecular function transferase activity nucleotide binding nucleotidyltransferase activity kinase activity protein binding catalytic activity ATP binding pantetheine-phosphate adenylyltransferase activity dephospho-CoA kinase activity Cellular component mitochondrial outer membrane mitochondrial matrix mitochondrion extracellular exosome cytoplasm nucleoplasm cytosol Biological process phosphorylation biosynthesis coenzyme A biosynthetic process metabolism Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 80347 71743 Ensembl ENSG00000068120 ENSMUSG00000001755 UniProt Q13057 Q9DBL7 RefSeq (mRNA) NM_001042529 NM_001042530 NM_001042531 NM_001042532 NM_025233 NM_027896 NM_001305982 RefSeq (protein) NP_001035994 NP_001035997 NP_079509 NP_001292911 Location (UCSC) Chr 17: 42.56 – 42.57 Mb Chr 11: 100.97 – 100.98 Mb PubMed search [3] [4]
Wikidata
View/Edit Human View/Edit Mouse

Function

COASY is an enzyme that catalyzes the last two steps in the synthesis of coenzyme A from vitamin B₅ (pantothenic acid). The primary substrate is 4'-phosphopantetheine and COASY is a bifunctional enzyme in this pathway:

• 4′-Phosphopantetheine is adenylated to form dephospho-CoA by the enzyme phosphopantetheine adenylyl-transferase (PPAT; CoaD)
• Next, dephospho-CoA is phosphorylated to coenzyme A by the enzyme dephospho-CoA kinase (DPCK; CoaE)

In mammals this is a single enzyme, but in organisms including yeast and bacteria these enzymes are encoded by separate genes.^[8]

Interactions

COASY has been shown to interact with P70-S6 Kinase 1.^[9] In 2009, COASY has also been implicated in PI3K signaling, as it was shown to interact with a regulatory subunit of PI3K.^[10]

Clinical significance

Loss of function mutations to COASY have been associated with an ultra-rare disease that causes neurodegeneration with brain iron accumulation called COASY protein-associated neurodegeneration (CoPAN), or NBIA6.^[8][11][12]