Cerebral organoids derived from Sandhoff disease-induced pluripotent stem cells exhibit impaired neurodifferentiation

Sandhoff disease, one of the GM2 gangliosidoses, is a lysosomal storage disorder characterized by the absence of β-hexosaminidase A and B activity and the concomitant lysosomal accumulation of its substrate, GM2 ganglioside. It features catastrophic neurodegeneration and death in early childhood. Ho...

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Detalles Bibliográficos
Autores principales: Allende, Maria L., Cook, Emily K., Larman, Bridget C., Nugent, Adrienne, Brady, Jacqueline M., Golebiowski, Diane, Sena-Esteves, Miguel, Tifft, Cynthia J., Proia, Richard L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The American Society for Biochemistry and Molecular Biology 2018
Materias:
Patient-Oriented and Epidemiological Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5832932/
https://www.ncbi.nlm.nih.gov/pubmed/29358305
http://dx.doi.org/10.1194/jlr.M081323

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5832932/
https://www.ncbi.nlm.nih.gov/pubmed/29358305
http://dx.doi.org/10.1194/jlr.M081323

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