Ceramidase

Ceramidase: Ceramidase 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]

Contents

1 Function

2 Clinical significance

3 References

4 External links

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 (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. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2614331.

External links

MeSH Ceramidase

EC 3.5.1.23

v · d · eHydrolases: carbon-nitrogen non-peptide (EC 3.5)

3.5.1: Linear amides /
Amidohydrolases
Asparaginase · Glutaminase · Urease · Biotinidase · Aspartoacylase · Ceramidase · Aspartylglucosaminidase · Fatty acid amide hydrolase · Histone deacetylase (Sirtuin)

3.5.2: Cyclic amides/
Amidohydrolases
Barbiturase · Beta-lactamase

3.5.3: Linear amidines/
Ureohydrolases
Arginase · Agmatinase · Protein-arginine deiminase

3.5.4: Cyclic amidines/
Aminohydrolases
Guanine deaminase · Adenosine deaminase · AMP deaminase · Inosine monophosphate synthase · DCMP deaminase · GTP cyclohydrolase I · Cytidine deaminase (AICDA, Activation-Induced (Cytidine) Deaminase)

3.5.5: Nitriles/
Aminohydrolases
Nitrilase

3.5.99: Other
Riboflavinase · Thiaminase II

B enzm: 1.1/2/3/4/5/6/7/8/10/11/13/14/15-18, 2.1/2/3/4/5/6/7/8, 2.7.10, 2.7.11-12, 3.1/2/3/4/5/6/7, 3.1.3.48, 3.4.21/22/23/24, 4.1/2/3/4/5/6, 5.1/2/3/4/99, 6.1-3/4/5-6

v · Metabolism: lipid metabolism · glycolipid enzymes

Sphingolipid

To glycosphingolipid

Glycosyltransferase · Sulfotransferase

To ceramide

From ganglioside: Beta-galactosidase · Hexosaminidase A · Neuraminidase · Glucocerebrosidase

From globoside: Hexosaminidase B · Alpha-galactosidase · Beta-galactosidase · Glucocerebrosidase

From sphingomyelin: Sphingomyelin phosphodiesterase (Sphingomyelin phosphodiesterase 1)
From sulfatide: Arylsulfatase A · Galactosylceramidase

To sphingosine

Ceramidase (ACER1, ACER2, ACER3, ASAH1, ASAH2, ASAH2B, ASAH2C)

Other

Sphingosine kinase

NCL
Palmitoyl protein thioesterase · Tripeptidyl peptidase I · CLN3 · CLN5 · CLN6 · CLN8

Ceramide synthesis
Serine C-palmitoyltransferase (SPTLC1) · Ceramide glucosyltransferase (UGCG)

M: MET

mt, k, c/g/r/p/y/i, f/h/s/l/o/e, a/u, n, m

k, cgrp/y/i, f/h/s/l/o/e, au, n, m, epon

m(A16/C10),i(k, c/g/r/p/y/i, f/h/s/o/e, a/u, n, m)

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