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 language | English (US) |
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Pages (from-to) | 294-304 |
Number of pages | 11 |
Journal | Mitochondrion |
Volume | 12 |
Issue number | 2 |
DOIs | |
State | Published - Mar 2012 |
Keywords
- Dilated cardiomyopathy
- Electron transport chain
- Gene knockout
- Mitochondria
- Oxidative phosphorylation
ASJC Scopus subject areas
- Molecular Medicine
- Molecular Biology
- Cell Biology