Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide

Stable Identifier
R-HSA-1606564
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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Galactocerebrosidase (GALC) hydrolyses the galactosyl moiety from galactocerebroside (also called galactosylceramide, GalCer) to form ceramide (Sakai et al. 1994). Defects in GALC are the cause of leukodystrophy globoid cell (GLD) (MIM:245200), also called Krabbe disease (Wenger et al. 1997).

Literature References
PubMed ID Title Journal Year
9338580 Molecular genetics of Krabbe disease (globoid cell leukodystrophy): diagnostic and clinical implications

Wenger, DA, Rafi, MA, Luzi, P

Hum Mutat 1997
7852280 Purification and characterization of galactocerebrosidase from human lymphocytes

Sakai, N, Inui, K, Midorikawa, M, Okuno, Y, Ueda, S, Iwamatsu, A, Okada, S

J Biochem 1994
Participants
Participates
Catalyst Activity

galactosylceramidase activity of GALC [lysosomal lumen]

Orthologous Events
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