Source:http://www.reactome.org/biopax/48887BiochemicalReaction4634
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Acid ceramidase (ASAH1) is a lysosomal enzyme that catalyses the hydrolysis of ceramide to sphingosine and free fatty acid. It functions as a heterodimer of one alpha and one beta subunit (Bernardo et al. 1995). Defects in ASAH1 are the cause of Farber lipogranulomatosis (FL) (MIM:228000), also called Farber disease (FD) (Zhang et al. 2000, Koch et al. 1996).,
Authored: Jassal, B, 2011-09-23,
Edited: Jassal, B, 2011-09-23,
Reviewed: Stephan, R, 2011-10-31
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Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome)
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3.5.1.23
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