COASY

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.{{cite journal | vauthors = Evers C, Seitz A, Assmann B, Opladen T, Karch S, Hinderhofer K, Granzow M, Paramasivam N, Eils R, Diessl N, Bartram CR, Moog U | display-authors = 6 | title = Diagnosis of CoPAN by whole exome sequencing: Waking up a sleeping tiger's eye | journal = American Journal of Medical Genetics. Part A | volume = 173 | issue = 7 | pages = 1878–1886 | date = July 2017 | pmid = 28489334 | doi = 10.1002/ajmg.a.38252 | s2cid = 27153945 }}

COASY has been shown to interact with P70-S6 Kinase 1.{{cite journal | vauthors = Nemazanyy I, Panasyuk G, Zhyvoloup A, Panayotou G, Gout IT, Filonenko V | title = Specific interaction between S6K1 and CoA synthase: a potential link between the mTOR/S6K pathway, CoA biosynthesis and energy metabolism | journal = FEBS Letters | volume = 578 | issue = 3 | pages = 357–362 | date = December 2004 | pmid = 15589845 | doi = 10.1016/j.febslet.2004.10.091 | s2cid = 9916948 | doi-access = free }}

In 2009, COASY has also been implicated in PI3K signaling, as it was shown to interact with a regulatory subunit of PI3K.{{cite journal | vauthors = Breus O, Panasyuk G, Gout IT, Filonenko V, Nemazanyy I | title = CoA synthase is in complex with p85alphaPI3K and affects PI3K signaling pathway | journal = Biochemical and Biophysical Research Communications | volume = 385 | issue = 4 | pages = 581–585 | date = August 2009 | pmid = 19482007 | doi = 10.1016/j.bbrc.2009.05.102 }}

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.{{cite journal | vauthors = van Dijk T, Ferdinandusse S, Ruiter JP, Alders M, Mathijssen IB, Parboosingh JS, Innes AM, Meijers-Heijboer H, Poll-The BT, Bernier FP, Wanders RJ, Lamont RE, Baas F | display-authors = 6 | title = Biallelic loss of function variants in COASY cause prenatal onset pontocerebellar hypoplasia, microcephaly, and arthrogryposis | journal = European Journal of Human Genetics | volume = 26 | issue = 12 | pages = 1752–1758 | date = December 2018 | pmid = 30089828 | doi = 10.1038/s41431-018-0233-0 | pmc = 6244412 }}{{cite web |title=OMIM Entry - #615643 - NEURODEGENERATION WITH BRAIN IRON ACCUMULATION 6; NBIA6 |url=https://www.omim.org/entry/615643 |website=www.omim.org |access-date=21 April 2022 |language=en-us}}

{{reflist}}

• {{UCSC gene info|COASY}}
• {{UCSC gene info|PPAT}}

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