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 language | English |
|---|---|
| Pages (from-to) | 26-39 |
| Number of pages | 14 |
| Journal | Brain Research |
| Volume | 1462 |
| DOIs | |
| Publication status | Published - 2012 Jun 26 |
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All Science Journal Classification (ASJC) codes
- Neuroscience(all)
- Molecular Biology
- Clinical Neurology
- Developmental Biology
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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 journal › Review article
TY - JOUR
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.
PY - 2012/6/26
Y1 - 2012/6/26
N2 - 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.
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.
UR - http://www.scopus.com/inward/record.url?scp=84862118369&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84862118369&partnerID=8YFLogxK
U2 - 10.1016/j.brainres.2012.04.031
DO - 10.1016/j.brainres.2012.04.031
M3 - Review article
C2 - 22608070
AN - SCOPUS:84862118369
VL - 1462
SP - 26
EP - 39
JO - Brain Research
JF - Brain Research
SN - 0006-8993
ER -