Mitochondrial transcription factor A is necessary for mtDNA maintance and embryogenesis in mice View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

1998-03

AUTHORS

Nils-Göran Larsson, Jianming Wang, Hans Wilhelmsson, Anders Oldfors, Pierre Rustin, Mark Lewandoski, Gregory S. Barsh, David A. Clayton

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. More... »

PAGES

231-236

Identifiers

URI

http://scigraph.springernature.com/pub.10.1038/ng0398-231

DOI

http://dx.doi.org/10.1038/ng0398-231

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1037709004

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/9500544


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