GLB1 hydrolyses a glycosaminoglycan

Stable Identifier
R-HSA-1630306
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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General
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Beta-galactosidase (GLB1) can cleave terminal galactose residues from glycosaminoglycans such as keratan sulfate (KS) (Asp et al. 1969). Defects in GLB1 cause the lysosomal storage diseases GM1gangliosidosis (Yoshida et al. 1991) and Morquio syndrome type B (Oshima et al. 1991).

Literature References
PubMed ID Title Journal Year
1928092 Human beta-galactosidase gene mutations in morquio B disease

Oshima, A, Yoshida, K, Shimmoto, M, Fukuhara, Y, Sakuraba, H, Suzuki, Y

Am J Hum Genet 1991
5822067 Human small-intestinal beta-galactosidases. Separation and characterization of one lactase and one hetero beta-galactosidase

Asp, NG, Dahlqvist, A, KoldovskĆ½, O

Biochem J 1969
1907800 Human beta-galactosidase gene mutations in GM1-gangliosidosis: a common mutation among Japanese adult/chronic cases

Yoshida, K, Oshima, A, Shimmoto, M, Fukuhara, Y, Sakuraba, H, Yanagisawa, N, Suzuki, Y

Am J Hum Genet 1991
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
beta-galactosidase activity of BGAL [lysosomal lumen]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created