Ontology highlight
ABSTRACT:
SUBMITTER: Lu J
PROVIDER: S-EPMC6588399 | biostudies-literature | 2019 Jun
REPOSITORIES: biostudies-literature
Lu Jiayin J Periz Goran G Lu Yu-Ning YN Tang Qing Q Liu Yang Y Zhang Tao T Shah Yajas Y Thombre Ravi R Aljumaah Reham R Li Weixin W Mojsilovic-Petrovic Jelena J Ji Yon Y Johnson Kenji K Kalb Robert R Wang Jiou J
Nature neuroscience 20190506 6
Misfolded protein toxicity and failure of protein quality control underlie neurodegenerative diseases including amyotrophic lateral sclerosis and frontotemporal dementia. Here, we identified Lethal(3)malignant brain tumor-like protein 1 (L3MBTL1) as a key regulator of protein quality control, the loss of which protected against the proteotoxicity of mutant Cu/Zn superoxide dismutase or C9orf72 dipeptide repeat proteins. L3MBTL1 acts by regulating p53-dependent quality control systems that degrad ...[more]