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- W2149384382 abstract "Hitherto, membralin has been a protein of unknown function. Here, we show that membralin mutant mice manifest a severe and early-onset motor neuron disease in an autosomal recessive manner, dying by postnatal day 5-6. Selective death of lower motor neurons, including those innervating the limbs, intercostal muscles, and diaphragm, is predominantly responsible for this fatal phenotype. Neural expression of a membralin transgene completely rescues membralin mutant mice. Mechanistically, we show that membralin interacts with Erlin2, an endoplasmic reticulum (ER) membrane protein that is located in lipid rafts and known to be important in ER-associated protein degradation (ERAD). Accordingly, the degradation rate of ERAD substrates is attenuated in cells lacking membralin. Membralin mutations or deficiency in mouse models induces ER stress, rendering neurons more vulnerable to cell death. Our study reveals a critical role of membralin in motor neuron survival and suggests a novel mechanism for early-onset motor neuron disease." @default.
- W2149384382 created "2016-06-24" @default.
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- W2149384382 date "2015-05-15" @default.
- W2149384382 modified "2023-10-05" @default.
- W2149384382 title "The critical role of membralin in postnatal motor neuron survival and disease" @default.
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- W2149384382 doi "https://doi.org/10.7554/elife.06500" @default.
- W2149384382 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/4460860" @default.
- W2149384382 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/25977983" @default.
- W2149384382 hasPublicationYear "2015" @default.
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