ASAH1 hydrolyzes ceramide

Stable Identifier
R-HSA-1606602
Type
Reaction [transition]
Species
Homo sapiens
Compartment
ReviewStatus
5/5
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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; Gebai et al, 2018). The reaction is stimulated by Saposin D (Sap D, PSAP(405-486)) (Tatti et al, 1999). 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).
Literature References
PubMed ID Title Journal Year
29692406 Structural basis for the activation of acid ceramidase

Nagar, B, Li, Z, Illes, K, Gorelik, A, Gebai, A

Nat Commun 2018
10406958 Structural and membrane-binding properties of saposin D

Vaccaro, AM, Andolfo, A, Amoresano, A, Salvioli, R, Tatti, M, Pucci, P, Ciaffoni, F

Eur J Biochem 1999
10993717 Human acid ceramidase gene: novel mutations in Farber disease

Mukherjee, AB, Zimonjic, D, Zhang, Z, Mandal, AK, Moser, A, Popescu, N, Moser, H, Mital, A

Mol Genet Metab 2000
8955159 Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase. Identification Of the first molecular lesion causing Farber disease

Bernardo, K, Koch, J, Levran, O, Schuchman, EH, Desnick, RJ, Quintern, LE, Gärtner, S, Sandhoff, K, Li, CM, Schnabel, D

J Biol Chem 1996
7744740 Purification, characterization, and biosynthesis of human acid ceramidase

Bernardo, K, Zenk, T, Ferlinz, K, Hurwitz, R, Schuchman, EH, Desnick, RJ, Sandhoff, K

J Biol Chem 1995
Participants
Participates
Catalyst Activity

N-acylsphingosine amidohydrolase activity of ASAH1 [lysosomal lumen]

This event is regulated
Orthologous Events
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