Ceramidase

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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]

External links[edit]