Open AccessLoss of Tmem106b leads to cerebellum Purkinje cell death and motor deficits
Author(s) -
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,
CastanedesCasey 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
Publication year - 2021
Publication title -
brain pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.986
H-Index - 132
eISSN - 1750-3639
pISSN - 1015-6305
DOI - 10.1111/bpa.12945
Subject(s) - purkinje cell , cerebellum , neuroscience , motor function , biology , medicine , physical medicine and rehabilitation
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.