Modelling brain diseases in mice: the challenges of design and analysis
作者： Kei Watase , Huda Y. Zoghbi
DOI: 10.1038/NRG1045
关键词:
摘要: Genetically engineered mice have been generated to model a variety of neurological disorders. Several these models provided valuable insights into the pathogenesis relevant diseases; however, they rarely reproduced all, or even most, features observed in corresponding human conditions. Here, we review challenges that must be faced when attempting accurately reproduce brain disorders mice, and discuss some ways overcome them. Building on knowledge gathered from study existing mutants, recent progress phenotyping mutant anticipate better methods for preclinical interventional trials significant advances understanding treatment diseases.
nature.com
sci-hub.se 参考文章(149)
ZHONG-XUN ZHOU, CHOI-IOK WONG, MADHABANANDA SAR, ELIZABETH M. WILSON, The androgen receptor: an overview. Recent Progress in Hormone Research. ,vol. 49, pp. 249- 274 ,(1994) , 10.1016/B978-0-12-571149-4.50017-9
R Charles Scriver, The Metabolic and Molecular Bases of Inherited Disease ,(1995)
Hélène Puccio, Delphine Simon, Mireille Cossée, Paola Criqui-Filipe, Francesco Tiziano, Judith Melki, Colette Hindelang, Robert Matyas, Pierre Rustin, Michel Koenig, Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits. Nature Genetics. ,vol. 27, pp. 181- 186 ,(2001) , 10.1038/84818
Minghua Chen, Victor O. Ona, Mingwei Li, Robert J. Ferrante, Klaus B. Fink, Shan Zhu, Jie Bian, Lei Guo, Laurie A. Farrell, Steve M. Hersch, Wendy Hobbs, Jean-Paul Vonsattel, Jang-Ho J. Cha, Robert M. Friedlander, Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease Nature Medicine. ,vol. 6, pp. 797- 801 ,(2000) , 10.1038/77528
Richard Z. Chen, Schahram Akbarian, Matthew Tudor, Rudolf Jaenisch, Deficiency of methyl-CpG binding protein-2 in CNS neurons results in a Rett-like phenotype in mice Nature Genetics. ,vol. 27, pp. 327- 331 ,(2001) , 10.1038/85906
James F. Gusella, Marcy E. MacDonald, Molecular genetics: Unmasking polyglutamine triggers in neurodegenerative disease Nature Reviews Neuroscience. ,vol. 1, pp. 109- 115 ,(2000) , 10.1038/35039051
Brian J. Bacskai, Stephen T. Kajdasz, Richard H. Christie, Cordelia Carter, Dora Games, Peter Seubert, Dale Schenk, Bradley T. Hyman, Imaging of amyloid-beta deposits in brains of living mice permits direct observation of clearance of plaques with immunotherapy. Nature Medicine. ,vol. 7, pp. 369- 372 ,(2001) , 10.1038/85525
H. Brent Clark, Eric N. Burright, Wael S. Yunis, Seth Larson, Claire Wilcox, Boyd Hartman, Antoni Matilla, Huda Y. Zoghbi, Harry T. Orr, Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations. The Journal of Neuroscience. ,vol. 17, pp. 7385- 7395 ,(1997) , 10.1523/JNEUROSCI.17-19-07385.1997
Jamel Chelly, Jean-Louis Mandel, Monogenic causes of X-linked mental retardation Nature Reviews Genetics. ,vol. 2, pp. 669- 680 ,(2001) , 10.1038/35088558
Dexiang Chen, Ryan L. Endres, Cherie A. Erickson, Kathleen F. Weis, Martha W. McGregor, Yoshihiro Kawaoka, Lendon G. Payne, Epidermal immunization by a needle-free powder delivery technology: immunogenicity of influenza vaccine and protection in mice. Nature Medicine. ,vol. 6, pp. 1187- 1190 ,(2000) , 10.1038/80538