Distinct effects of tafazzin deletion in differentiated and undifferentiated mitochondria

Mitochondrion. 2009 Apr;9(2):86-95. doi: 10.1016/j.mito.2008.12.001. Epub 2008 Dec 11.


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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • 1-Acylglycerophosphocholine O-Acyltransferase / genetics*
  • Acyltransferases
  • Animals
  • Cardiolipins / metabolism
  • Drosophila Proteins / genetics*
  • Drosophila melanogaster
  • Electron Microscope Tomography
  • Gene Deletion
  • Mice
  • Mitochondria / ultrastructure*
  • Mitochondrial Membranes / ultrastructure
  • Transcription Factors / genetics*


  • Cardiolipins
  • Drosophila Proteins
  • Transcription Factors
  • Acyltransferases
  • tafazzin protein, mouse
  • 1-Acylglycerophosphocholine O-Acyltransferase
  • tafazzin protein, Drosophila