Disease Animal Models of TDP-43 Proteinopathy and Their Pre-Clinical Applications

International Journal of Molecular Sciences

Yu-Chih Liu¹  Po-Min Chiang¹ 

[1] Institute of Clinical Medicine, National Cheng Kung University, Tainan 704, Taiwan; E-Mails:

关键词: amyotrophic lateral sclerosis;    disease models;    frontotemperal lobar degeneration;    proteinopathy;    TDP-43;    therapy;   

DOI  :  10.3390/ijms141020079

来源: mdpi

【 摘 要 】

Frontotemperal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) are two common neurodegenerative diseases. TDP-43 is considered to be a major disease protein in FTLD/ALS, but it’s exact role in the pathogenesis and the effective treatments remains unknown. To address this question and to determine a potential treatment for FTLD/ALS, the disease animal models of TDP-43 proteinopathy have been established. TDP-43 proteinopathy is the histologic feature of FTLD/ALS and is associated with disease progression. Studies on the disease animal models with TDP-43 proteinopathy and their pre-clinical applications are reviewed and summarized. Through these disease animal models, parts of TDP-43 functions in physiological and pathological conditions will be better understood and possible treatments for FTLD/ALS with TDP-43 proteinopathy may be identified for possible clinical applications in the future.

【 授权许可】

CC BY   
© 2013 by the authors; licensee MDPI, Basel, Switzerland