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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...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Dove Medical Press
2016
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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 |