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Mechanisms of distribution of mouse β-galactosidase in the adult GM1-gangliosidosis brain

✍ Scribed by Broekman, M L D; Tierney, L A; Benn, C; Chawla, P; Cha, J H; Sena-Esteves, M


Book ID
109855088
Publisher
Nature Publishing Group
Year
2008
Tongue
English
Weight
326 KB
Volume
16
Category
Article
ISSN
0969-7128

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G(M1)-gangliosidosis is a lysosomal storage disorder caused by a deficiency of beta-galactosidase (GLB1). The GLB1 gene gives rise to the GLB1 lysosomal enzyme and to the elastin binding protein (EBP), involved in elastic fiber deposition. GLB1 forms a complex with protective protein cathepsin A (PP