Abnormal differentiation of Sandhoff disease model mouse-derived multipotent stem cells toward a neural lineage

In Sandhoff disease (SD), the activity of the lysosomal hydrolytic enzyme, β-hexosaminidase (Hex), is lost due to a Hexb gene defect, which results in the abnormal accumulation of the substrate, GM2 ganglioside (GM2), in neuronal cells, causing neuronal loss, microglial activation, and astrogliosis....

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Detalles Bibliográficos
Autores principales: Ogawa, Yasuhiro, Kaizu, Katsutoshi, Yanagi, Yusuke, Takada, Subaru, Sakuraba, Hitoshi, Oishi, Kazuhiko
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2017
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5456357/
https://www.ncbi.nlm.nih.gov/pubmed/28575132
http://dx.doi.org/10.1371/journal.pone.0178978

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5456357/
https://www.ncbi.nlm.nih.gov/pubmed/28575132
http://dx.doi.org/10.1371/journal.pone.0178978

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