COASY
Protein-coding gene in mammals
From Wikipedia, the free encyclopedia
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]
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| 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 | ||||||||||||||||||||||||||||||||||||||||||||||||||
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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]
