期刊
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
卷 1832, 期 9, 页码 1421-1436出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.bbadis.2013.03.012
关键词
ALS; Models; TDP-43; FUS; SOD1; VCP
资金
- MRC [G0801110, MR/K000608/1, G0500288] Funding Source: UKRI
- Medical Research Council [MR/K000608/1, G0801110, G0500288] Funding Source: researchfish
- Medical Research Council [MR/K000608/1, G0801110, G0500288] Funding Source: Medline
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterised by the degeneration of upper and lower motor neurons. Recent advances in our understanding of some of the genetic causes of ALS, such as mutations in SOD1, TARDBP, FUS and VCP have led to the generation of rodent models of the disease, as a strategy to help our understanding of the pathophysiology of ALS and to assist in the development of therapeutic strategies. This review provides detailed descriptions of TDP-43, PUS and VCP models of ALS, and summarises potential therapeutics which have been recently trialled in rodent models of the disease. This article is part of a Special Issue entitled: Animal Models of Disease. (C) 2013 Elsevier B.V. All rights reserved.
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