acid ceramidase; acylsphingosine deacylase; N-acylsphingosine amidohydrolase; AC; 32 kD heart protein; PHP32 [Contains: acid ceramidase subunit alpha; acid ceramidase subunit beta] (ASAH1, ASAH)

Function

• hydrolyzes the sphingolipid ceramide into sphingosine + free fatty acid.

N-acylsphingosine + H2O <--> carboxylate + sphingosine

Structure

• heterodimer of one alpha & one beta subunit
• belongs to the acid ceramidase family

Compartment

lysosome

Expression

broadly expressed, highest expression in heart

Pathology

• defects in ASAH1 are the cause of Farber disease

More general terms

• hydrolase
• glycoprotein
• multisubunit protein

Database

• OMIM: https://mirror.omim.org/entry/228000
• UniProt: http://www.uniprot.org/uniprot/Q13510.html