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Insights into post-translational processing of β-galactosidase in an animal model resembling late infantile human G(M1)-gangliosidosis
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 inf...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Blackwell Publishing Ltd
2008
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3918082/ https://www.ncbi.nlm.nih.gov/pubmed/18088383 http://dx.doi.org/10.1111/j.1582-4934.2007.00204.x |