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).
Koldovský, O, Asp, NG, Dahlqvist, A
Fukuhara, Y, Yoshida, K, Suzuki, Y, Sakuraba, H, Oshima, A, Shimmoto, M
Fukuhara, Y, Yoshida, K, Suzuki, Y, Sakuraba, H, Oshima, A, Yanagisawa, N, Shimmoto, M
beta-galactosidase activity of BGAL [lysosomal lumen]
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