RGD Reference Report - Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice. - Rat Genome Database

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

Authors: Larsson, NG  Wang, J  Wilhelmsson, H  Oldfors, A  Rustin, P  Lewandoski, M  Barsh, GS  Clayton, DA 
Citation: Larsson NG, etal., Nat Genet. 1998 Mar;18(3):231-6.
RGD ID: 1578538
Pubmed: PMID:9500544   (View Abstract at PubMed)
DOI: DOI:10.1038/ng0398-231   (Journal Full-text)

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.

Objects referenced in this article
Gene Tfam transcription factor A, mitochondrial Rattus norvegicus

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