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Impaired Neural Differentiation of Induced Pluripotent Stem Cells Generated from a Mouse Model of Sandhoff Disease

Sandhoff disease (SD) is a glycosphingolipid storage disease that arises from mutations in the Hexb gene and the resultant deficiency in β-hexosaminidase activity. This deficiency results in aberrant lysosomal accumulation of the ganglioside GM2 and related glycolipids, and progressive deterioration...

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Detalles Bibliográficos
Autores principales: Ogawa, Yasuhiro, Tanaka, Makoto, Tanabe, Miho, Suzuki, Toshihiro, Togawa, Tadayasu, Fukushige, Tomoko, Kanekura, Takuro, Sakuraba, Hitoshi, Oishi, Kazuhiko
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3561340/
https://www.ncbi.nlm.nih.gov/pubmed/23383290
http://dx.doi.org/10.1371/journal.pone.0055856