期刊
NEUROSCIENCE
卷 189, 期 -, 页码 100-107出版社
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.neuroscience.2011.05.031
关键词
TAF1; DYT3 dystonia; striosome; neurodegeneration; transcription dysregulation syndrome
资金
- Ministry of Education, Culture, Sports, Science and Technology of Japan [20591025, 2139026900]
- United States National Institutes of Health [P50 NS38372, R37 HD028341]
- Grants-in-Aid for Scientific Research [21390269, 23500428, 20591025] Funding Source: KAKEN
The neuron-specific isoform of the TAF1 gene (N-TAF1) is thought to be involved in the pathogenesis of DYT3 dystonia, which leads to progressive neurodegeneration in the striatum. To determine the expression pattern of N-TAF1 transcripts, we developed a specific monoclonal antibody against the N-TAF1 protein. Here we show that in the rat brain, N-TAF1 protein appears as a nuclear protein within subsets of neurons in multiple brain regions. Of particular interest is that in the striatum, the nuclei possessing N-TAF1 protein are largely within medium spiny neurons, and they are distributed preferentially, though not exclusively, in the striosome compartment. The compartmental preference and cell type-selective distribution of N-TAF1 protein in the striatum are strikingly similar to the patterns of neuronal loss in the striatum of DYT3 patients. Our findings suggest that the distribution of N-TAF1 protein could represent a key molecular characteristic contributing to the pattern of striatal degeneration in DYT3 dystonia. (C) 2011 IBRO. Published by Elsevier Ltd. All rights reserved.
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