Search on: ACID CERAMIDASE 
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Descriptor English:   Acid Ceramidase 
Descriptor Spanish:   ceramidasa ácida 
Descriptor Portuguese:   Ceramidase Ácida 
Tree Number:   D08.811.277.087.250.100
Definition English:   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. 
Entry Combination - descr/qualif English:   Acid Ceramidase/deficiency use Farber Lipogranulomatosis
 
History Note English:   2009(1982); use GALACTOSYLGALACTOSYLGLUCOSYLCERAMIDASE 1983-2008 
Allowable Qualifiers English:  
AD administration & dosage AE adverse effects
AN analysis AI antagonists & inhibitors
BI biosynthesis BL blood
CF cerebrospinal fluid CS chemical synthesis
CH chemistry CL classification
DE drug effects EC economics
GE genetics HI history
IM immunology IP isolation & purification
ME metabolism PK pharmacokinetics
PD pharmacology PH physiology
PO poisoning RE radiation effects
ST standards SD supply & distribution
TU therapeutic use TO toxicity
UL ultrastructure UR urine
Record Number:   53113 
Unique Identifier:   D055573 

Occurrence in VHL:
 

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