期刊
MITOCHONDRION
卷 9, 期 2, 页码 86-95出版社
ELSEVIER SCI LTD
DOI: 10.1016/j.mito.2008.12.001
关键词
Tafazzin; Cardiolipin; Mitochondria; Barth syndrome; Electron microscopy; Electron tomography
资金
- National Institutes of Health [HL078788, HL083065, GM071044]
- Barth Syndrome Foundation
- United Mitochondrial Disease Foundation
- Prinses Beatrix Fonds [WAR05-0126]
- New York State Office of Science, Technology and Academic Research
- Div Of Molecular and Cellular Bioscience
- Direct For Biological Sciences [0741914] Funding Source: National Science Foundation
Tafazzin is a conserved mitochondrial protein that is required to maintain normal content and composition of cardiolipin. We used electron tomography to investigate the effect of tafazzin deletion on mitochondrial structure and found that cellular differentiation plays a crucial role in the manifestation of abnormalities. This conclusion was reached by comparing differentiated cardiomyocytes with embryonic stem cells from mouse and by comparing different tissues from Drosophila melanogaster. The data suggest that tafazzin deficiency affects cardiolipin in all mitochondria, but significant alterations of the ultrastructure, such as remodeling and aggregation of inner membranes, will only occur after specific differentiation. (C) 2008 Elsevier B.V. and Mitochondria Research Society. All rights reserved.
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