Article
Insights into post-translational processing of beta-galactosidase in an animal model resembling late infantile human G-gangliosidosis.
Journal of cellular and molecular medicine - 1 Jan 2000
Kreutzer R, Kreutzer M, Pröpsting M J, Sewell A C, Leeb T, Naim H Y, Baumgärtner W
Abstract excerpt
G(M1)-gangliosidosis is a lysosomal storage disorder caused by a deficiency of ss-galactosidase activity. Human GM1-gangliosidosis has been classified into three forms according to the age of clinical onset and specific biochemical parameters. In the present study, a canine model for type II late infantile human GM1-gangliosidosis was investigated 'in vitro' in detail. For a better understanding of the molecular...
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