期刊
NATURE MEDICINE
卷 20, 期 8, 页码 881-885出版社
NATURE PUBLISHING GROUP
DOI: 10.1038/nm.3617
关键词
-
资金
- Centro de Investigacion Biomedica en Red de Enfermedades Neurodegenerativas (CiberNed-Instituto de Salud Carlos III)
- Ministerio de Ciencia e Innovacion (MICINN)
- Ministerio de Economia y Competitividad (MINECO)
- Comunidad Autonoma de Madrid
- Fundacion Ramon Areces
- European Commission [278486]
- CSIC
An imbalance of tau isoforms containing either three or four microtubule-binding repeats causes frontotemporal dementia with parkinsonism linked to-chromosome 17 (FTDP-17) in families with intronic mutations in the MAPT gene. Here we report equivalent imbalances at the mRNA and protein levels and increased total tau levels in the brains of subjects with Huntington's disease (HD) together with rod-like tau deposits along neuronal nuclei. These tau nuclear rods show an ordered filamentous ultrastructure and can be found filling the neuronal nuclear indentations previously reported in HD brains. Finally, alterations in serine/arginine-rich splicing factor-6 coincide with tau missplicing, and a role of tau in HD pathogenesis is evidenced by the attenuation of motor abnormalities of mutant HTT transgenic mice in tau knockout backgrounds.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据