Mice deleted for heart-type cytochrome c oxidase subunit 7a1 develop dilated cardiomyopathy

Maik Hüttemann, Scott Klewer, Icksoo Lee, Alena Pecinova, Petr Pecina, Jenney Liu, Michael Lee, Jeffrey W. Doan, Douglas F Larson, Elise Slack, Bita Maghsoodi, Robert P. Erickson, Lawrence I. Grossman

Research output: Contribution to journalArticlepeer-review

31 Scopus citations

Abstract

Subunit 7a of mouse cytochrome c oxidase (Cox) displays a contractile muscle-specific isoform, Cox7a1, that is the major cardiac form. To gain insight into the role of this isoform, we have produced a new knockout mouse line that lacks Cox7a1. We show that homozygous and heterozygous Cox7a1 knockout mice, although viable, have reduced Cox activity and develop a dilated cardiomyopathy at 6. weeks of age. Surprisingly, the cardiomyopathy improves and stabilizes by 6. months of age. Cox7a1 knockout mice incorporate more of the "liver-type" isoform Cox7a2 into the cardiac Cox holoenzyme and, also surprisingly, have higher tissue ATP levels.

Original languageEnglish (US)
Pages (from-to)294-304
Number of pages11
JournalMitochondrion
Volume12
Issue number2
DOIs
StatePublished - Mar 2012

Keywords

  • Dilated cardiomyopathy
  • Electron transport chain
  • Gene knockout
  • Mitochondria
  • Oxidative phosphorylation

ASJC Scopus subject areas

  • Molecular Medicine
  • Molecular Biology
  • Cell Biology

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