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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...
Những tác giả chính: | , , , , , |
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Định dạng: | Online Bài viết Texto |
Ngôn ngữ: | English |
Được phát hành: |
BioMed Central
2013
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Những chủ đề: | |
Truy cập trực tuyến: | 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 |