Int. J. Mol. Sci. 2013, 14(10), 20079-20111; doi:10.3390/ijms141020079
Review

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

1,2email, 1,2email and 1,2,* email
Received: 5 July 2013; in revised form: 13 September 2013 / Accepted: 18 September 2013 / Published: 9 October 2013
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract: 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.
Keywords: amyotrophic lateral sclerosis; disease models; frontotemperal lobar degeneration; proteinopathy; TDP-43; therapy
PDF Full-text Download PDF Full-Text [792 KB, uploaded 19 June 2014 05:11 CEST]

Export to BibTeX |
EndNote


MDPI and ACS Style

Liu, Y.-C.; Chiang, P.-M.; Tsai, K.-J. Disease Animal Models of TDP-43 Proteinopathy and Their Pre-Clinical Applications. Int. J. Mol. Sci. 2013, 14, 20079-20111.

AMA Style

Liu Y-C, Chiang P-M, Tsai K-J. Disease Animal Models of TDP-43 Proteinopathy and Their Pre-Clinical Applications. International Journal of Molecular Sciences. 2013; 14(10):20079-20111.

Chicago/Turabian Style

Liu, Yu-Chih; Chiang, Po-Min; Tsai, Kuen-Jer. 2013. "Disease Animal Models of TDP-43 Proteinopathy and Their Pre-Clinical Applications." Int. J. Mol. Sci. 14, no. 10: 20079-20111.


Int. J. Mol. Sci. EISSN 1422-0067 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert