TY - JOUR
T1 - Disease animal models of TDP-43 proteinopathy and their pre-clinical applications
AU - Liu, Yu Chih
AU - Chiang, Po Min
AU - Tsai, Kuen Jer
PY - 2013/10/9
Y1 - 2013/10/9
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=84885358733&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84885358733&partnerID=8YFLogxK
U2 - 10.3390/ijms141020079
DO - 10.3390/ijms141020079
M3 - Review article
C2 - 24113586
AN - SCOPUS:84885358733
SN - 1661-6596
VL - 14
SP - 20079
EP - 20111
JO - International journal of molecular sciences
JF - International journal of molecular sciences
IS - 10
ER -