| Mitofilin对线粒体功能和疾病发生的影响 |
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| 引用本文： | 况凌云,董士业,刘丹慧. Mitofilin对线粒体功能和疾病发生的影响[J]. 细胞生物学杂志, 2014, 0(4): 540-545 |
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| 作者姓名： | 况凌云 董士业 刘丹慧 |
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| 作者单位： | 温州医科大学检验医学院、生命科学学院,浙江省医学遗传学重点实验室,温州325035 |
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| 基金项目： | 国家自然科学基金青年基金（批准号：81301744）和浙江省公益性技术应用研究计划项目（批准号：2011C33041）资助的课题 |
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| 摘 要： | Mitofilin是一种线粒体内膜蛋白，与多种线粒体蛋白相互作用，共同参与线粒体内膜嵴形态的维持、线粒体内蛋白质的转运过程等。干扰mitofilin~表达不仅引起线粒体结构的异常，而且明显抑制了线粒体功能的正常发挥。最近的研究表明，在多种疾病中mitonlin都异常表达，从而导致线粒体结构的完整性和功能的障碍，促进了疾病的发生发展。
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| 关 键 词： | mitofilin 线粒体 嵴膜 蛋白转运 疾病 |
The Role of Mitofilin in the Regulation of Mitochondrial Functions and Its Implication in Diseases |
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| Kuang Linyun,Dong Shiye,Liu Danhui. The Role of Mitofilin in the Regulation of Mitochondrial Functions and Its Implication in Diseases[J]. Chinese Journal of Cell Biology, 2014, 0(4): 540-545 |
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| Authors: | Kuang Linyun Dong Shiye Liu Danhui |
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| Affiliation: | (School of Laboratory Medicine, and Life Sciences, Zhejiang Provincial Key Laboratory of Medical Genetics Wenzhou Medical College, Wenzhou 325035, China) |
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| Abstract: | Mitofilin, a mitochondrial inner membrane protein, is involved in many important biological pro- cesses such as the maintenance of mitochondrial cristae morphology, mitochondrial protein transport processes, etc. Alteration of mitofilin expression level does not only affect mitochondrial structure, but also signifcantly inhibits normal mitochondrial functions. Recent studies showed that mitofilin was abnormal expressed in a variety of diseases, leading to altered mitochondrial structural integrity and functions, and promoting the development of the diseases. |
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| Keywords: | mitofilin mitochondia cristae protein transport disease |
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