Ceramidase
Ceramidase (EC 3.5.1.23, acylsphingosine deacylase, glycosphingolipid ceramide deacylase) is an enzyme which cleaves fatty acids from ceramide, producing sphingosine (SPH) which in turn is phosphorylated by a sphingosine kinase to form sphingosine-1-phosphate (S1P).[1]
Function
Ceramide, SPH, and S1P are bioactive lipids that mediate cell proliferation, differentiation, apoptosis, adhesion, and migration. Presently, 7 human ceramidases encoded by 7 distinct genes have been cloned:[1]
- acid ceramidase (ASAH1) – cell survival
- neutral ceramidase (ASAH2, ASAH2B, ASAH2C) – protective against inflammatory cytokines
- alkaline ceramidase 1 (ACER1) – mediating cell differentiation by controlling the generation of SPH and S1P
- alkaline ceramidase 2 (ACER2) – important for cell proliferation and survival
- alkaline ceramidase 3 (ACER3)
Clinical significance
A deficiency in ASAH1 is associated with Farber disease.
References
- ^ a b Mao C, Obeid LM (2008). "Ceramidases: regulators of cellular responses mediated by ceramide, sphingosine, and sphingosine-1-phosphate". Biochim. Biophys. Acta. 1781 (9): 424–34. doi:10.1016/j.bbalip.2008.06.002. PMC 2614331. PMID 18619555.
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External links
- Ceramidase at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
- EC 3.5.1.23
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