Degenerative ataxias: genetics, pathogenesis and animal models.
Degenerative ataxias: genetics, pathogenesis and animal models.
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退行性共济失调：遗传学、发病机制和动物模型。
DOI：
10.1097/00019052-199708000-00002
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发表时间：
1997
影响因子：
4.8
通讯作者：
A. Kakizuka
中科院分区：
文献类型：
--
作者：
A. Kakizuka
目前，已确定七种遗传性神经退行性疾病是由相关基因中编码聚谷氨酰胺的 CAG 三联体扩展引起的。长链聚谷氨酰胺似乎在发病机制中发挥着关键作用。最近刚刚用培养细胞和动物建立的疾病模型将有助于阐明常见疾病机制和治疗方法。
At present, seven inherited neurodegenerative disorders have been identified to be caused by the polyglutamine-coding CAG triplet expansions in the genes responsible. The long stretch of polyglutamines appears to play a key role in the pathogenesis. Disease models, just recently established with cultured cells and animals, will enable elucidation of common disease mechanisms and treatments.