title: 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.
authors.affiliation: Skirball Institute, New York University Langone, Medical Center, New York, NY 10016, USA.
authors.forename: Devrim
authors.initials: D
authors.lastname: Acehan
authors.forename: Zaza
authors.initials: Z
authors.lastname: Khuchua
authors.forename: Riekelt H
authors.initials: RH
authors.lastname: Houtkooper
authors.forename: Ashim
authors.initials: A
authors.lastname: Malhotra
authors.forename: Johanna
authors.initials: J
authors.lastname: Kaufman
authors.forename: Frédéric M
authors.initials: FM
authors.lastname: Vaz
authors.forename: Mindong
authors.initials: M
authors.lastname: Ren
authors.forename: Howard A
authors.initials: HA
authors.lastname: Rockman
authors.forename: David L
authors.initials: DL
authors.lastname: Stokes
authors.forename: Michael
authors.initials: M
authors.lastname: Schlame
mesh_terms: D008243:1-Acylglycerophosphocholine O-Acyltransferase / Q000235:genetics*; D000217:Acyltransferases; D000818:Animals; D002308:Cardiolipins / Q000378:metabolism; D029721:Drosophila Proteins / Q000235:genetics*; D004331:Drosophila melanogaster; D055032:Electron Microscope Tomography; D017353:Gene Deletion; D051379:Mice; D008928:Mitochondria / Q000648:ultrastructure*; D051336:Mitochondrial Membranes / Q000648:ultrastructure; D014157:Transcription Factors / Q000235:genetics*
chemical_list: D002308:Cardiolipins; D029721:Drosophila Proteins; D014157:Transcription Factors; D000217:Acyltransferases; C486363:tafazzin protein, mouse; D008243:1-Acylglycerophosphocholine O-Acyltransferase; C514176:tafazzin protein, Drosophila