GLB1 hydrolyses a glycosaminoglycan

Stable Identifier
R-HSA-1630306
Type
Reaction
Species
Homo sapiens
Compartment
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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
5822067 Human small-intestinal beta-galactosidases. Separation and characterization of one lactase and one hetero beta-galactosidase

KoldovskĆ½, O, Asp, NG, Dahlqvist, A

Biochem J 1969
1928092 Human beta-galactosidase gene mutations in morquio B disease

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

Am J Hum Genet 1991
1907800 Human beta-galactosidase gene mutations in GM1-gangliosidosis: a common mutation among Japanese adult/chronic cases

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

Am J Hum Genet 1991
Participants
Participates
Catalyst Activity

beta-galactosidase activity of BGAL [lysosomal lumen]

Orthologous Events
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