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Acid Ceramidase

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Definition: A ceramidase subtype that is active at acid pH. It plays an important role in sphingolipid degradation by catalyzing the lysosomal hydrolysis of ceramide to sphingosine and free fatty acid. Inherited deficiency of acid ceramidase activity results in FARBER LIPOGRANULOMATOSIS.     
 
SubstanceCAS Registry & nameCategoriesSourceDrugs*
Asah1 protein, rat  EC 3.5.1.23   *Acid Ceramidase.
Asah1 protein, mouse  EC 3.5.1.23   *Acid Ceramidase.
ASAH1 protein, human  EC 3.5.1.23   *Acid Ceramidase Farber Lipogranulomatosis.

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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