Ceramidase

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Ceramidase
Identifiers
EC number3.5.1.23
CAS number37289-06-8
Databases
IntEnzIntEnz view
BRENDABRENDA entry
ExPASyNiceZyme view
KEGGKEGG entry
MetaCycmetabolic pathway
PRIAMprofile
PDB structuresRCSB PDB PDBe PDBsum

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[edit]

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[edit]

A deficiency in ASAH1 is associated with Farber disease.

References[edit]

  1. ^ a b Mao C, Obeid LM (September 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.

External links[edit]