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

作者：Liu Yu Chih; Chiang Po Min; Tsai Kuen Jer^*

来源：International Journal of Molecular Sciences, 2013, 14(10): 20079-20111.

DOI：10.3390/ijms141020079

• amyotrophic lateral sclerosis
• disease models
• frontotemperal lobar degeneration
• proteinopathy
• TDP-43
• therapy

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%26apos;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.

• 出版日期2013-10