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

Research output: Contribution to journalReview article

74 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)26-39
Number of pages14
JournalBrain Research
Volume1462
DOIs
Publication statusPublished - 2012 Jun 26

Fingerprint

RNA-Binding Proteins
Rodentia
Genetic Association Studies
Motor Neurons
Missense Mutation
Cause of Death
Research Personnel
RNA
Mutation
Therapeutics
Genes
Direction compound

All Science Journal Classification (ASJC) codes

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

Cite this

Tsao, W., Jeong, Y. H., Lin, S., Ling, J., Price, D. L., Chiang, P. M., & Wong, P. C. (2012). Rodent models of TDP-43: Recent advances. Brain Research, 1462, 26-39. https://doi.org/10.1016/j.brainres.2012.04.031
Tsao, William ; Jeong, Yun Ha ; Lin, Sophie ; Ling, Jonathan ; Price, Donald L. ; Chiang, Po Min ; Wong, Philip C. / Rodent models of TDP-43 : Recent advances. In: Brain Research. 2012 ; Vol. 1462. pp. 26-39.
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Tsao, W, Jeong, YH, Lin, S, Ling, J, Price, DL, Chiang, PM & Wong, PC 2012, 'Rodent models of TDP-43: Recent advances', Brain Research, vol. 1462, pp. 26-39. https://doi.org/10.1016/j.brainres.2012.04.031

Rodent models of TDP-43 : Recent advances. / Tsao, William; Jeong, Yun Ha; Lin, Sophie; Ling, Jonathan; Price, Donald L.; Chiang, Po Min; Wong, Philip C.

In: Brain Research, Vol. 1462, 26.06.2012, p. 26-39.

Research output: Contribution to journalReview article

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T1 - Rodent models of TDP-43

T2 - Recent advances

AU - Tsao, William

AU - Jeong, Yun Ha

AU - Lin, Sophie

AU - Ling, Jonathan

AU - Price, Donald L.

AU - Chiang, Po Min

AU - Wong, Philip C.

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AB - 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.

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Tsao W, Jeong YH, Lin S, Ling J, Price DL, Chiang PM et al. Rodent models of TDP-43: Recent advances. Brain Research. 2012 Jun 26;1462:26-39. https://doi.org/10.1016/j.brainres.2012.04.031