Disease animal models of TDP-43 proteinopathy and their pre-clinical applications.
Int J Mol Sci
; 14(10): 20079-111, 2013 Oct 09.
Article
en En
| MEDLINE
| ID: mdl-24113586
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.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Proteínas de Unión al ADN
/
Proteinopatías TDP-43
Límite:
Animals
/
Humans
Idioma:
En
Revista:
Int J Mol Sci
Año:
2013
Tipo del documento:
Article
País de afiliación:
Taiwán