@article{cec3f05d451f4e2b988d937a92dce1df,
title = "Disease animal models of TDP-43 proteinopathy and their pre-clinical applications",
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.",
author = "Liu, {Yu Chih} and Chiang, {Po Min} and Tsai, {Kuen Jer}",
year = "2013",
month = oct,
day = "9",
doi = "10.3390/ijms141020079",
language = "English",
volume = "14",
pages = "20079--20111",
journal = "International journal of molecular sciences",
issn = "1661-6596",
publisher = "Multidisciplinary Digital Publishing Institute (MDPI)",
number = "10",
}