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Letter

Nature Genetics 37, 974–979 (1 September 2005) | doi:10.1038/ng1620

Protein accumulation and neurodegeneration in the woozy mutant mouse is caused by disruption of SIL1, a cochaperone of BiP

Lihong Zhao , Chantal Longo-Guess , Belinda S Harris , Jeong-Woong Lee & Susan L Ackerman

Endoplasmic reticulum (ER) chaperones and ER stress have been implicated in the pathogenesis of neurodegenerative disorders, such as Alzheimer and Parkinson diseases, but their contribution to neuron death remains uncertain. In this study, we establish a direct in vivo link between ER dysfunction and neurodegeneration.