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Distinct effects of tafazzin deletion in differentiated and undifferentiated mitochondria.

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.

SUBMITTER: Acehan D

PROVIDER: S-EPMC2660382 | biostudies-other | 2009 Apr

REPOSITORIES: biostudies-other

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Distinct effects of tafazzin deletion in differentiated and undifferentiated mitochondria.

Acehan Devrim D Khuchua Zaza Z Houtkooper Riekelt H RH Malhotra Ashim A Kaufman Johanna J Vaz Frédéric M FM Ren Mindong M Rockman Howard A HA Stokes David L DL Schlame Michael M

Mitochondrion 20081211 2

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 ...[more]

PMID: 19114128

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