Genetically modified rodent models of SCA17.
Genetically modified rodent models of SCA17.
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DOI：
10.1002/jnr.23984
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发表时间：
2017-08
影响因子：
4.2
通讯作者：
Li S
中科院分区：
文献类型：
--
作者：
Cui Y;Yang S;Li XJ;Li S
Spinocerebellar ataxia type 17 (SCA17) is a family member of Autosomal Dominant Cerebellar Ataxias (ADCA) characterized by variable manifestations, including cerebellar ataxia, dementia and psychiatric symptoms. Since the identification of a CAG repeat expansion in the TBP gene in a patient with ataxia in 1999 and then verification of this expansion in SCA17 patients in 2001, several SCA17 rodent models, including both knock-in and transgenic models in mouse and rat, have been established to explore the phenotypic features and pathogenesis of SCA17. These animal models revealed different pathologic changes and phenotypes that are associated with the expression of mutant TBP protein and the CAG repeat lengths. It is important to understand how mutant TBP can cause differential pathological events in SCA17 animal models. In this review, we summarize and compare these animal models for the nature of transgenes and their expression as well as phenotypical features. We also discuss potential directions to use them for future studies.
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影响因子：
16.2
作者：
Yang S;Huang S;Gaertig MA;Li XJ;Li S
通讯作者：
Li S
影响因子：
14.5
作者：
Fujigasaki, H;Martin, JJ;Brice, A
通讯作者：
Brice, A
影响因子：
9.9
作者：
Maltecca, F;Filla, A;De Michele, G
通讯作者：
De Michele, G
影响因子：
13.8
作者：
ROEDER, RG
通讯作者：
ROEDER, RG
影响因子：
4.8
作者：
Friedman, Meyer J.;Wang, Chuan-En;Li, Shihua
通讯作者：
Li, Shihua