Rodent models of TDP-43: Recent advances

William Tsao, Yun Ha Jeong, Sophie Lin, Jonathan Ling, Donald L. Price, Po Min Chiang, Philip C. Wong

研究成果: Review article同行評審

84 引文 斯高帕斯(Scopus)

摘要

Recently, missense mutations in the gene TARDBP encoding TDP-43 have been linked to familial ALS. The discovery of genes encoding these RNA binding proteins, such as TDP-43 and FUS/TLS, raised the notion that altered RNA metabolism is a major factor underlying the pathogenesis of ALS. To begin to unravel how mutations in TDP-43 cause dysfunction and death of motor neurons, investigators have employed both gain- and loss-of-function studies in rodent model systems. Here, we will summarize major findings from the initial sets of TDP-43 transgenic and knockout rodent models, identify their limitations, and point to future directions toward clarification of disease mechanism(s) and testing of therapeutic strategies that ultimately may lead to novel therapy for this devastating disease. This article is part of a Special Issue entitled RNA-Binding Proteins.

原文English
頁(從 - 到)26-39
頁數14
期刊Brain Research
1462
DOIs
出版狀態Published - 2012 六月 26

All Science Journal Classification (ASJC) codes

  • Neuroscience(all)
  • Molecular Biology
  • Clinical Neurology
  • Developmental Biology

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