Mito-mice: animal models for mitochondrial DNA-based diseases.
Semin Cell Dev Biol
; 12(6): 459-65, 2001 Dec.
Article
en En
| MEDLINE
| ID: mdl-11735380
ABSTRACT
We have successfully produced "Mito-mice" harbouring a pathogenic mtDNA mutation. We generated the mice by introducing mitochondria with a 4696 base-pair mtDNA deletion (Delta mtDNA4696) into mouse embryos. This deletion encompasses nucleotides 7759-12 454 and includes six tRNA genes and seven structural genes. In Mito-mice, the Delta mtDNA4696 is transmitted maternally, and induces mitochondrial dysfunction in various tissues. Most of the Mito-mice with high proportions of the Delta mtDNA4696 died at about age 6 months due to renal failure. Mito-mice are the first animal model for mtDNA-based diseases and will be valuable for studying pathogenesis and for identifying effective drug and gene therapies.
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Base de datos:
MEDLINE
Asunto principal:
ADN Mitocondrial
/
Modelos Animales
/
Enfermedades Mitocondriales
Idioma:
En
Revista:
Semin Cell Dev Biol
Asunto de la revista:
EMBRIOLOGIA
Año:
2001
Tipo del documento:
Article