Loss of Tmem106b leads to cerebellum Purkinje cell death and motor deficits. (11th March 2021)

• Record Type: Journal Article
• Authors: Rademakers, Rosa; Nicholson, Alexandra M.; Ren, Yingxue; Koga, Shunsuke; Nguyen, Hung Phuoc; Brooks, Mieu; Qiao, Wenhui; Quicksall, Zachary S.; Matchett, Billie; Perkerson, Ralph B.; Kurti, Aishe; Castanedes‐Casey, Monica; Phillips, Virginia; Librero, Ariston L; Fernandez De Castro, Cristhoper H.; Baker, Matthew C.; Roemer, Shanu F.; Murray, Melissa E.; Asmann, Yan; Fryer, John D.; Bu, Guojun; Dickson, Dennis W.; Zhou, Xiaolai
• Is Part Of: Brain pathology. Volume 31:Number 3(2021)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2021-03-11
• Subjects: Nervous system -- Diseases -- Periodicals; Brain -- Diseases -- Periodicals; Neurology -- Periodicals; Brain Diseases -- Periodicals; Cerveau -- Maladies -- Périodiques; Système nerveux -- Maladies -- Périodiques; Neurologie -- Périodiques; 616.805
• DOI: 10.1111/bpa.12945
• Languages: English
• ISSNs: 1015-6305

Abstract

Abstract : 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.