Mitofilin对线粒体功能和疾病发生的影响  

The Role of Mitofi lin in the Regulation of Mitochondrial Functions and Its Implication in Diseases

作　　者：况凌云[1] 董士业 刘丹慧[1] 

机构地区：[1]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

出　　处：《中国细胞生物学学报》2014年第4期540-545,共6页Chinese Journal of Cell Biology

基　　金：国家自然科学基金青年基金(批准号:81301744);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~~

摘　　要：Mitofi lin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mitofi lin的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mitofi lin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。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.

关 键 词：mitofilin 线粒体 嵴膜 蛋白转运 疾病 

分 类 号：R363[医药卫生—病理学] Q244[医药卫生—基础医学]