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Loss of Tmem106b leads to cerebellum Purkinje cell death and motor deficits
Brain Pathology ( IF 6.4 ) Pub Date : 2021-03-11 , DOI: 10.1111/bpa.12945
Rosa Rademakers 1, 2, 3 , Alexandra M Nicholson 1 , Yingxue Ren 4 , Shunsuke Koga 1 , Hung Phuoc Nguyen 1 , Mieu Brooks 1 , Wenhui Qiao 1 , Zachary S Quicksall 4 , Billie Matchett 1 , Ralph B Perkerson 1 , Aishe Kurti 1 , Monica Castanedes-Casey 1 , Virginia Phillips 1 , Ariston L Librero 1 , Cristhoper H Fernandez De Castro 1 , Matthew C Baker 1 , Shanu F Roemer 1 , Melissa E Murray 1 , Yan Asmann 4 , John D Fryer 1 , Guojun Bu 1 , Dennis W Dickson 1 , Xiaolai Zhou 1, 5
Affiliation  

TMEM106B has been recently implicated in multiple neurodegenerative diseases. Here, Rademakers et al. report a late-onset cerebellar Purkinje cell loss and progressive decline in motor function and gait deficits in a conventional Tmem106b−/− mouse model. By using high-power microscopy and bulk RNA sequencing, the authors further identify lysosomal and immune dysfunction as potential underlying mechanisms of the Purkinje cell loss.
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中文翻译:

Tmem106b 缺失导致小脑浦肯野细胞死亡和运动障碍

TMEM106B 最近与多种神经退行性疾病有关。在这里,Rademakers 等人。报告了传统Tmem106b-/-小鼠模型中迟发性小脑浦肯野细胞丢失和运动功能和步态缺陷的进行性下降。通过使用高倍显微镜和大量 RNA 测序,作者进一步确定溶酶体和免疫功能障碍是浦肯野细胞丢失的潜在潜在机制。
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更新日期:2021-03-11
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