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

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 a...

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Main Authors:	Liu, Yu-Chih, Chiang, Po-Min, Tsai, Kuen-Jer
Format:	Online
Language:	English
Published:	Molecular Diversity Preservation International (MDPI) 2013
Online Access:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3821604/

id	pubmed-3821604
recordtype	oai_dc
spelling	pubmed-38216042013-11-11 Disease Animal Models of TDP-43 Proteinopathy and Their Pre-Clinical Applications Liu, Yu-Chih Chiang, Po-Min Tsai, Kuen-Jer Review 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. Molecular Diversity Preservation International (MDPI) 2013-10-09 /pmc/articles/PMC3821604/ /pubmed/24113586 http://dx.doi.org/10.3390/ijms141020079 Text en © 2013 by the authors; licensee MDPI, Basel, Switzerland http://creativecommons.org/licenses/by/3.0/ This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution license (http://creativecommons.org/licenses/by/3.0/).
repository_type	Open Access Journal
institution_category	Foreign Institution
institution	US National Center for Biotechnology Information
building	NCBI PubMed
collection	Online Access
language	English
format	Online
author	Liu, Yu-Chih Chiang, Po-Min Tsai, Kuen-Jer
spellingShingle	Liu, Yu-Chih Chiang, Po-Min Tsai, Kuen-Jer Disease Animal Models of TDP-43 Proteinopathy and Their Pre-Clinical Applications
author_facet	Liu, Yu-Chih Chiang, Po-Min Tsai, Kuen-Jer
author_sort	Liu, Yu-Chih
title	Disease Animal Models of TDP-43 Proteinopathy and Their Pre-Clinical Applications
title_short	Disease Animal Models of TDP-43 Proteinopathy and Their Pre-Clinical Applications
title_full	Disease Animal Models of TDP-43 Proteinopathy and Their Pre-Clinical Applications
title_fullStr	Disease Animal Models of TDP-43 Proteinopathy and Their Pre-Clinical Applications
title_full_unstemmed	Disease Animal Models of TDP-43 Proteinopathy and Their Pre-Clinical Applications
title_sort	disease animal models of tdp-43 proteinopathy and their pre-clinical applications
description	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.
publisher	Molecular Diversity Preservation International (MDPI)
publishDate	2013
url	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3821604/
_version_	1612024777327771648

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

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