Ontology highlight
ABSTRACT:
SUBMITTER: Flores BN
PROVIDER: S-EPMC6499398 | biostudies-literature | 2019 Apr
REPOSITORIES: biostudies-literature
Flores Brittany N BN Li Xingli X Malik Ahmed M AM Martinez Jose J Beg Asim A AA Barmada Sami J SJ
Cell reports 20190401 4
The majority of individuals with amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) exhibit neuronal cytoplasmic inclusions rich in the RNA binding protein TDP43. Even so, the relation between the RNA binding properties of TDP43 and neurodegeneration remains obscure. Here, we show that engineered mutations disrupting a salt bridge between the RNA recognition motifs of TDP43 interfere with RNA binding and eliminate the recognition of native TDP43 substrates. The same mutations ...[more]