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Animal models of GM2 gangliosidosis: utility and limitations

GM2 gangliosidosis, a subset of lysosomal storage disorders, is caused by a deficiency of the glycohydrolase, β-N-acetylhexosaminidase, and includes the closely related Tay–Sachs and Sandhoff diseases. The enzyme deficiency prevents the normal, stepwise degradation of ganglioside, which accumulates...

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Detalles Bibliográficos
Autores principales: Lawson, Cheryl A, Martin, Douglas R
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Dove Medical Press 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4959762/
https://www.ncbi.nlm.nih.gov/pubmed/27499644
http://dx.doi.org/10.2147/TACG.S85354