Distinct effects of tafazzin deletion in differentiated and undifferentiated mitochondria |
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| Authors: | Devrim Acehan Zaza Khuchua Riekelt H Houtkooper Ashim Malhotra Johanna Kaufman Frédéric M Vaz Mindong Ren Howard A Rockman David L Stokes Michael Schlame |
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| Affiliation: | 1. Centre d''Etudes Supérieures de la Rennaissance, Université François Rabelais, Tours, France;2. Centre de Référence Maladies Neuromusculaires Nantes-Angers, CHU de Nantes, Nantes, France;3. Atlantic Gene Therapy Institute, Nantes, France;1. Department of Anesthesiology and Cell Biology, New York University School of Medicine, New York, NY 10016, USA;2. Department of Biological Sciences, Wayne State University, Detroit, MI 48202, USA |
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| Abstract: | 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. |
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