GLB1 hydrolyzes SapB/C:LacCer

Stable Identifier
R-HSA-1606312
Type
Reaction [transition]
Species
Homo sapiens
Compartment
ReviewStatus
5/5
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Lactosylceramide (LacCer) is the central intermediate in glycosphingolipid catabolism. Beta-galactosidase (GLB1) cleaves the galactose moiety from LacCer to form glucosylceramide. LacCer accumulates in GLB1 deficiencies (MIM: 611458) like GM1-gangliosidosis but also in prosaposin deficiency (PSAPD, MIM: 611721), which shows saposin cofactors are essential for the reaction (Asp et al. 1969; Bradova et al., 1993). We follow Sandhoff & Sandhoff (2018), who state that Saposins B/C bind to LacCer.

Participants
Participates
Catalyst Activity

beta-galactosidase activity of BGAL [lysosomal lumen]

Orthologous Events
Authored
Reviewed
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