Gimap3 regulates tissue-specific mitochondrial DNA segregation.
PLoS Genet
; 6(10): e1001161, 2010 Oct 14.
Article
em En
| MEDLINE
| ID: mdl-20976251
ABSTRACT
Mitochondrial DNA (mtDNA) sequence variants segregate in mutation and tissue-specific manners, but the mechanisms remain unknown. The segregation pattern of pathogenic mtDNA mutations is a major determinant of the onset and severity of disease. Using a heteroplasmic mouse model, we demonstrate that Gimap3, an outer mitochondrial membrane GTPase, is a critical regulator of this process in leukocytes. Gimap3 is important for T cell development and survival, suggesting that leukocyte survival may be a key factor in the genetic regulation of mtDNA sequence variants and in modulating human mitochondrial diseases.
Texto completo:
1
Coleções:
01-internacional
Temas:
Geral
Base de dados:
MEDLINE
Assunto principal:
Haplótipos
/
DNA Mitocondrial
/
Proteínas de Ligação ao GTP
/
Proteínas Mitocondriais
/
GTP Fosfo-Hidrolases
/
Proteínas de Membrana
Tipo de estudo:
Prognostic_studies
Limite:
Animals
/
Female
/
Humans
/
Male
Idioma:
En
Revista:
PLoS Genet
Assunto da revista:
GENETICA
Ano de publicação:
2010
Tipo de documento:
Article
País de afiliação:
Finlândia