Deficiency in the mouse mitochondrial adenine nucleotide translocator isoform 2 gene is associated with cardiac noncompaction
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- 作者：Jason E. Kokoszkaa ; Katrina G. Waymireb ; Adrian Flierlc ; Katelyn M. Sweeneyd ; Alessia Angelind ; Grant R. MacGregorb ; Douglas C. Wallaced ; wallaced1@email.chop.edu" class="auth_mail" title="E-mail the corresponding author
- 关键词：Mitochondria ; Heart ; Cardiomyopathy ; Adenine nucleotide translocator 2 (ANT2) ; Hypertrabeculation ; Noncompaction
- 刊名：Biochimica et Biophysica Acta (BBA) - Bioenergetics
- 出版年：2016
- 出版时间：August 2016
- 年：2016
- 卷：1857
- 期：8
- 页码：1203-1212
- 全文大小：1931 K
文摘
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The ANTs exchange ATP/ADP across the mitochondrial inner membrane and regulate the mtPTP.
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There are several ANT isoforms, ANT1 and ANT2 being expressed in both fetal and adult heart.
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Inactivation of the mouse Ant1 gene permits normal cardiac development but results in adult hypertrophic cardiomyopathy.
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Inactivation of the mouse Ant2 gene is embryonic lethal associated with cardiac hypertrabeculation/noncompaction.
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The differential cardiac toxicity of Ant1 & Ant2 inactivation is hypothesized as differential regulation of the mtPTP.