Article
Dendritic spinopathy in transgenic mice expressing ALS/dementia-linked mutant UBQLN2.
Proceedings of the National Academy of Sciences of the United States of America - 7 Oct 2014
Gorrie George H, Fecto Faisal, Radzicki Daniel, Weiss Craig, Shi Yong, Dong Hongxin, Zhai Hong, Fu Ronggen, Liu Erdong, Li Sisi, Arrat Hasan, Bigio Eileen H, Disterhoft John F, Martina Marco, Mugnaini Enrico, Siddique Teepu, Deng Han-Xiang
Abstract excerpt
Mutations in the gene encoding ubiquilin2 (UBQLN2) cause amyotrophic lateral sclerosis (ALS), frontotemporal type of dementia, or both. However, the molecular mechanisms are unknown. Here, we show that ALS/dementia-linked UBQLN2(P497H) transgenic mice develop neuronal pathology with ubiquilin2/ubiquitin/p62-positive inclusions in the brain, especially in the hippocampus, recapitulating several key pathological...
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