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

Mice deleted for heart-type cytochrome c oxidase subunit 7a1 develop dilated cardiomyopathy.
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DOI:
10.1016/j.mito.2011.11.002
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发表时间:
2012-03
期刊:
影响因子:
4.4
通讯作者:
Grossman, Lawrence I.
Grossman, Lawrence I.
中科院分区:
生物学3区
文献类型:
--
作者:
Huettemann, Maik;Klewer, Scott;Lee, Icksoo;Pecinova, Alena;Pecina, Petr;Liu, Jenney;Lee, Michael;Doan, Jeffrey W.;Larson, Douglas;Slack, Elise;Maghsoodi, Bita;Erickson, Robert P.;Grossman, Lawrence I.

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小鼠细胞色素c氧化酶(考克斯)的亚基7a显示出收缩性肌肉特异性同种型,Cox7a1,这是主要的心脏形式。为了深入了解这种亚型的作用,我们已经产生了一种新的敲除小鼠品系,缺乏Cox7a1。我们发现,纯合子和杂合子Cox7a1基因敲除小鼠,虽然可行,但考克斯活性降低,并在6周龄时发展为扩张型心肌病。令人惊讶的是,心肌病在6个月大时得到改善和稳定。Cox7a1基因敲除小鼠将更多的“肝脏型”同种型Cox7a2整合到心脏考克斯全酶中,并且还令人惊讶地具有更高的组织ATP水平。
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.
DOI: 10.1111/j.1742-4658.2007.06093.x
发表时间: 2007-11-01
期刊: FEBS JOURNAL
影响因子: 5.4
作者:
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通讯作者: Grossman, Lawrence I.
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