Insufficient ER-stress response causes selective mouse cerebellar granule cell degeneration resembling that seen in congenital disorders of glycosylation

BACKGROUND: Congenital disorders of glycosylation (CDGs) are inherited diseases caused by glycosylation defects. Incorrectly glycosylated proteins induce protein misfolding and endoplasmic reticulum (ER) stress. The most common form of CDG, PMM2-CDG, is caused by deficiency in the cytosolic enzyme p...

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Detalles Bibliográficos
Autores principales: Sun, Liangwu, Zhao, Yingjun, Zhou, Kun, Freeze, Hudson H, Zhang, Yun-wu, Xu, Huaxi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2013
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3907076/
https://www.ncbi.nlm.nih.gov/pubmed/24305089
http://dx.doi.org/10.1186/1756-6606-6-52

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