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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Bibliografske podrobnosti
izdano v:PLoS One
Main Authors: Ogawa, Yasuhiro, Kaizu, Katsutoshi, Yanagi, Yusuke, Takada, Subaru, Sakuraba, Hitoshi, Oishi, Kazuhiko
Format: Artigo
Jezik:Inglês
Izdano: Public Library of Science 2017
Teme:
Research Article
Online dostop:https://ncbi.nlm.nih.gov/pmc/articles/PMC5456357/
https://ncbi.nlm.nih.gov/pubmed/28575132
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0178978
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