title: Mice deleted for heart-type cytochrome c oxidase subunit 7a1 develop dilated cardiomyopathy.
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.
authors: Center for Molecular Medicine and Genetics, Wayne State University School of Medicine, Detroit, MI 48201, USA.
authors: Maik
authors: M
authors: Hüttemann
authors: Scott
authors: S
authors: Klewer
authors: Icksoo
authors: I
authors: Lee
authors: Alena
authors: A
authors: Pecinova
authors: Petr
authors: P
authors: Pecina
authors: Jenney
authors: J
authors: Liu
authors: Michael
authors: M
authors: Lee
authors: Jeffrey W
authors: JW
authors: Doan
authors: Douglas
authors: D
authors: Larson
authors: Elise
authors: E
authors: Slack
authors: Bita
authors: B
authors: Maghsoodi
authors: Robert P
authors: RP
authors: Erickson
authors: Lawrence I
authors: LI
authors: Grossman
mesh_terms: D000818:Animals; D002311:Cardiomyopathy, Dilated / Q000235:genetics*; D003576:Electron Transport Complex IV / Q000235:genetics* / Q000378:metabolism*; D017353:Gene Deletion; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D009206:Myocardium / Q000473:pathology*; D021122:Protein Subunits / Q000235:genetics; D016019:Survival Analysis
chemical_list: D021122:Protein Subunits; C514296:Cox7a2 protein, mouse; D003576:Electron Transport Complex IV; C573492:cytochrome c oxidase subunit 7a1, mouse