Biology:COASY
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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.[1][2][3]
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.[4]
Interactions
COASY has been shown to interact with P70-S6 Kinase 1.[5] In 2009, COASY has also been implicated in PI3K signaling, as it was shown to interact with a regulatory subunit of PI3K.[6]
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.[4][7][8]
References
- ↑ "Complete reconstitution of the human coenzyme A biosynthetic pathway via comparative genomics". The Journal of Biological Chemistry 277 (24): 21431–21439. June 2002. doi:10.1074/jbc.M201708200. PMID 11923312.
- ↑ "Molecular cloning of CoA Synthase. The missing link in CoA biosynthesis". The Journal of Biological Chemistry 277 (25): 22107–22110. June 2002. doi:10.1074/jbc.C200195200. PMID 11980892.
- ↑ "Entrez Gene: COASY Coenzyme A synthase". https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=80347.
- ↑ 4.0 4.1 "Diagnosis of CoPAN by whole exome sequencing: Waking up a sleeping tiger's eye". American Journal of Medical Genetics. Part A 173 (7): 1878–1886. July 2017. doi:10.1002/ajmg.a.38252. PMID 28489334.
- ↑ "Specific interaction between S6K1 and CoA synthase: a potential link between the mTOR/S6K pathway, CoA biosynthesis and energy metabolism". FEBS Letters 578 (3): 357–362. December 2004. doi:10.1016/j.febslet.2004.10.091. PMID 15589845.
- ↑ "CoA synthase is in complex with p85alphaPI3K and affects PI3K signaling pathway". Biochemical and Biophysical Research Communications 385 (4): 581–585. August 2009. doi:10.1016/j.bbrc.2009.05.102. PMID 19482007.
- ↑ "Biallelic loss of function variants in COASY cause prenatal onset pontocerebellar hypoplasia, microcephaly, and arthrogryposis". European Journal of Human Genetics 26 (12): 1752–1758. December 2018. doi:10.1038/s41431-018-0233-0. PMID 30089828.
- ↑ "OMIM Entry - #615643 - NEURODEGENERATION WITH BRAIN IRON ACCUMULATION 6; NBIA6" (in en-us). https://www.omim.org/entry/615643.
External links
- Human COASY genome location and COASY gene details page in the UCSC Genome Browser.
- Human PPAT genome location and PPAT gene details page in the UCSC Genome Browser.
Further reading
- "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides". Gene 138 (1–2): 171–174. January 1994. doi:10.1016/0378-1119(94)90802-8. PMID 8125298.
- "A 100-kb physical and transcriptional map around the EDH17B2 gene: identification of three novel genes and a pseudogene of a human homologue of the rat PRL-1 tyrosine phosphatase". Human Genetics 96 (5): 532–538. November 1995. doi:10.1007/bf00197407. PMID 8529999.
- "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library". Gene 200 (1–2): 149–156. October 1997. doi:10.1016/S0378-1119(97)00411-3. PMID 9373149.
- "Identification and characterization of the gene encoding the human phosphopantetheine adenylyltransferase and dephospho-CoA kinase bifunctional enzyme (CoA synthase)". The Biochemical Journal 365 (Pt 1): 13–18. July 2002. doi:10.1042/BJ20020569. PMID 11994049.
- "Subcellular localization and regulation of coenzyme A synthase". The Journal of Biological Chemistry 278 (50): 50316–50321. December 2003. doi:10.1074/jbc.M307763200. PMID 14514684.
- "Large-scale characterization of HeLa cell nuclear phosphoproteins". Proceedings of the National Academy of Sciences of the United States of America 101 (33): 12130–12135. August 2004. doi:10.1073/pnas.0404720101. PMID 15302935. Bibcode: 2004PNAS..10112130B.
- "Specific interaction between S6K1 and CoA synthase: a potential link between the mTOR/S6K pathway, CoA biosynthesis and energy metabolism". FEBS Letters 578 (3): 357–362. December 2004. doi:10.1016/j.febslet.2004.10.091. PMID 15589845.
- "Towards a proteome-scale map of the human protein-protein interaction network". Nature 437 (7062): 1173–1178. October 2005. doi:10.1038/nature04209. PMID 16189514. Bibcode: 2005Natur.437.1173R.
- "Transcriptome analysis of human gastric cancer". Mammalian Genome 16 (12): 942–954. December 2005. doi:10.1007/s00335-005-0075-2. PMID 16341674.
- "Identification of a novel CoA synthase isoform, which is primarily expressed in the brain". Biochemical and Biophysical Research Communications 341 (4): 995–1000. March 2006. doi:10.1016/j.bbrc.2006.01.051. PMID 16460672.
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