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

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

1
Institute of Clinical Medicine, National Cheng Kung University, Tainan 704, Taiwan
2
Institute of Basic Medical Science, National Cheng Kung University, Tainan 704, Taiwan
*
Author to whom correspondence should be addressed.
Received: 5 July 2013 / Revised: 13 September 2013 / Accepted: 18 September 2013 / Published: 9 October 2013
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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. View Full-Text
Keywords: amyotrophic lateral sclerosis; disease models; frontotemperal lobar degeneration; proteinopathy; TDP-43; therapy amyotrophic lateral sclerosis; disease models; frontotemperal lobar degeneration; proteinopathy; TDP-43; therapy
This is an open access article distributed under the Creative Commons Attribution License (CC BY 3.0).

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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.

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