GLB1 hydrolyzes SapB/C:LacCer

Stable Identifier
R-HSA-1606312
Type
Reaction [transition]
Species
Homo sapiens
Compartment
lysosomal lumen
ReviewStatus
5/5
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GLB1 hydrolyzes SapB/C:LacCer
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The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout
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.
Literature References
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Output
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Catalyst Activity

beta-galactosidase activity of BGAL [lysosomal lumen]

Physical Entity
BGAL [lysosomal lumen] (Homo sapiens)
Activity
beta-galactosidase activity (GO:0004565)
Orthologous Events
GLB1 hydrolyzes SapB/C:LacCer (Bos taurus)
GLB1 hydrolyzes SapB/C:LacCer (Caenorhabditis elegans)
GLB1 hydrolyzes SapB/C:LacCer (Canis familiaris)
GLB1 hydrolyzes SapB/C:LacCer (Dictyostelium discoideum)
GLB1 hydrolyzes SapB/C:LacCer (Drosophila melanogaster)
GLB1 hydrolyzes SapB/C:LacCer (Gallus gallus)
GLB1 hydrolyzes SapB/C:LacCer (Mus musculus)
GLB1 hydrolyzes SapB/C:LacCer (Rattus norvegicus)
GLB1 hydrolyzes SapB/C:LacCer (Sus scrofa)
Authored
Jassal, B  (2011-09-21)
Reviewed
Stephan, R  (2011-10-31)
Created
Jassal, B  (2011-09-21)
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