Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice.
Nat Genet
; 18(3): 231-6, 1998 Mar.
Article
em En
| MEDLINE
| ID: mdl-9500544
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.
Buscar no Google
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Fatores de Transcrição
/
Proteínas Virais
/
DNA Mitocondrial
/
Proteínas Nucleares
/
Regulação da Expressão Gênica no Desenvolvimento
/
Proteínas Mitocondriais
/
Proteínas de Ligação a DNA
/
Morte Fetal
Limite:
Animals
Idioma:
En
Revista:
Nat Genet
Assunto da revista:
GENETICA MEDICA
Ano de publicação:
1998
Tipo de documento:
Article
País de afiliação:
Suécia