Gutiérrez-Aguilar Manuel, Douglas Diana L, Gibson Anne K, Domeier Timothy L, Molkentin Jeffery D, Baines Christopher P
Dalton Cardiovascular Research Center, University of Missouri-Columbia, Columbia, MO 65211, USA.
Department of Medical Pharmacology and Physiology, University of Missouri-Columbia, Columbia, MO 65211, USA.
J Mol Cell Cardiol. 2014 Jul;72:316-25. doi: 10.1016/j.yjmcc.2014.04.008. Epub 2014 Apr 21.
The Mitochondrial Permeability Transition (MPT) pore is a voltage-sensitive unselective channel known to instigate necrotic cell death during cardiac disease. Recent models suggest that the isomerase cyclophilin D (CypD) regulates the MPT pore by binding to either the F0F1-ATP synthase lateral stalk or the mitochondrial phosphate carrier (PiC). Here we confirm that CypD, through its N-terminus, can directly bind PiC. We then generated cardiac-specific mouse strains overexpressing or with decreased levels of mitochondrial PiC to assess the functionality of such interaction. While PiC overexpression had no observable pathologic phenotype, PiC knockdown resulted in cardiac hypertrophy along with decreased ATP levels. Mitochondria isolated from the hearts of these mouse lines and their respective non-transgenic controls had no divergent phenotype in terms of oxygen consumption and Ca(2+)-induced MPT, as assessed by swelling and Ca(2+)-retention measurements. These results provide genetic evidence indicating that the mitochondrial PiC is not a critical component of the MPT pore.
线粒体通透性转换(MPT)孔是一种电压敏感性非选择性通道,已知在心脏疾病期间会引发坏死性细胞死亡。最近的模型表明,异构酶亲环蛋白D(CypD)通过与F0F1 - ATP合酶侧柄或线粒体磷酸盐载体(PiC)结合来调节MPT孔。在此我们证实,CypD通过其N末端可直接结合PiC。然后我们构建了过表达或线粒体PiC水平降低的心脏特异性小鼠品系,以评估这种相互作用的功能。虽然PiC过表达没有可观察到的病理表型,但PiC敲低导致心脏肥大并伴有ATP水平降低。通过肿胀和钙保留测量评估,从这些小鼠品系及其各自的非转基因对照心脏中分离出的线粒体在氧消耗和钙诱导的MPT方面没有不同的表型。这些结果提供了遗传学证据,表明线粒体PiC不是MPT孔的关键组成部分。