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- Title
基于诱导性多潜能干细胞的亨廷顿舞蹈病发病机制研究.
- Authors
刘涛; 刘文超; 刘新秀; 邓敏; 陈静
- Abstract
Objective: The cells from Huntington disease (HD) patient and healthy people were induced to differentiate into motor neurons based on pluripotency characteristic of iPSC. and the motor neurons was used to explore the pathogenesis of HD. Methods: The iPS cells collected from HD patient and healthy people were differentiated into motor neurons under the action of certain growth factors and neurotrophic factors. The expression levels of motor neuron specific markers HB9 and ISL1 were evaluated by immunofluorescence staining. Reactive oxygen species and mitochondrial membrane potential of control and HD patient motor neurons were evaluated by flow cytometry, and DCFH-DA and JC-1 were used as the fluorescent probes respectively. Results: Successfully differentiated HD patient and control specific iPS cells into motor neurons after 25 days. And immunofluorescence staining showed that (3111-tubulin positive neurons express both motor neuron specific markers HB9 and ISL1. Furthermore, the ROS levels fluorescence intensity of HD patient motor neurons (4704.33± 390.50) had significantly enhanced, than controls! 2840.33± 166.20 )(P=0.002), on behalf of the mitochondrial membrane potential of fluorescence intensity red-green ratio (2.74± 0.13) had significantly reduced than controls (3.97± 0.29) (P=0.03). Conclusions: HD patient-specific iPSC' can be differentiated into motor neurons, and provided a model for experimental research. The pathogenesis of Huntington's disease was associated with mitochondrial dysfunction of motor neurons.
- Publication
Progress in Modern Biomedicine, 2016, Vol 16, Issue 33, p6418
- ISSN
1673-6273
- Publication type
Article
- DOI
10.13241/j.cnki.pmb.2016.33.005