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Nat Genet. 1998 Mar;18(3):231-6.

Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice.

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  • 1Department of Molecular Medicine, Center for Molecular Medicine L8:02, Stockholm, Sweden. nils-goran.larsson@cmm.ki.se

Abstract

The regulation of mitochondrial DNA (mtDNA) expression is crucial for mitochondrial biogenesis during development and differentiation. We have disrupted the mouse gene for mitochondrial transcription factor A (Tfam; formerly known as m-mtTFA) by gene targetting of loxP-sites followed by cre-mediated excision in vivo. Heterozygous knockout mice exhibit reduced mtDNA copy number and respiratory chain deficiency in heart. Homozygous knockout embryos exhibit a severe mtDNA depletion with abolished oxidative phosphorylation. Mutant embryos proceed through implantation and gastrulation, but die prior to embryonic day (E)10.5. Thus, Tfam is the first mammalian protein demonstrated to regulate mtDNA copy number in vivo and is essential for mitochondrial biogenesis and embryonic development.

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PMID:
9500544
[PubMed - indexed for MEDLINE]
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