TAR DNA binding protein-43 and fused in sarcoma/translocated in liposarcoma protein in two neurodegenerative diseases.
10.3881/j.issn.1000-503X.2012.03.020
- Author:
Xin-ning WANG
1
;
Li-ying CUI
Author Information
1. Department of Neurology, PUMC Hospital, CAMS and PUMC, Beijing 100730, China.
- Publication Type:Journal Article
- MeSH:
Amyotrophic Lateral Sclerosis;
DNA-Binding Proteins;
genetics;
metabolism;
Frontotemporal Dementia;
Humans;
Mutation;
RNA Processing, Post-Transcriptional;
RNA-Binding Protein FUS;
genetics;
metabolism
- From:
Acta Academiae Medicinae Sinicae
2012;34(3):286-292
- CountryChina
- Language:English
-
Abstract:
TAR DNA binding protein-43(TDP-43) and fused in sarcoma/translocated in liposarcoma protein (FUS/TLS) have been found to be associated with two neurodegenerative diseases - amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Mutations in TDP-43 and FUS/TLS lead to abnormal protein expressions, which result in altered RNA processing. The pathological changes of TDP-43 and FUS/TLS-associated ALS and FTD are similar. Although the interactions between ALS and FTD remain unknown, it is speculated that TDP-43 and FUS/TLS-associated neurodegenerative diseases may share similar pathogenesis.
