Journal
BIOCHEMICAL JOURNAL
Volume 416, Issue -, Pages 15-26Publisher
PORTLAND PRESS LTD
DOI: 10.1042/BJ20080847
Keywords
cytochrome b; mitochondrion; pentatricopeptide repeat (PPR) protein; pentatricopeptide repeat domain protein 2 (PTCD2); RNA processing
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Funding
- Canadian Institutes for Health Research [MT 6573]
- MitoMarch for Kirkland
- Association de Addose Lactique-Saguenay-Lac-Saint-Jean (Quebec)
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Mice homozygous for a defect in the PTCD2 (pentatricopeptide repeat domain protein 2) gene were generated in order to study the role of this protein in mitochondrial RNA metabolism. These mice displayed specific but variable reduction of ubiquinol-cytochronic c reductase complex activity in mitochondria of heart, liver and skeletal muscle due to a decrease in the expression of mitochondrial DNA-encoded cytochrome b, the catalytic core of the complex. This reduction in mitochondrial function has a profound effect on the myocardium, with replacement of ventricular cardiomyocytes by fibro-fatty tissue. Northern blotting showed a reduction in the mRNA for the mitochondrial DNA encoded proteins cytochrome b (cytb) and ND5 (NADH dehydrogenase subunit 5) and an elevation in a combined pre-processed ND5-CYTB transcript. This suggests that the PTCD2 protein is involved in processing RNA transcripts involving cytochrome b derived from mitochondrial DNA. This defines the site for PTCD2 action in mammalian mitochondria and suggests a possible role for dysfunction of this protein in the actiology of heart failure.
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