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Semin Cell Dev Biol. 2001 Dec;12(6):459-65.

Mito-mice: animal models for mitochondrial DNA-based diseases.

Author information

1
Institute of Biological Sciences, University of Tsukuba, Ibaraki 305-8572, Japan

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.

PMID:
11735380
DOI:
10.1006/scdb.2001.0283
[Indexed for MEDLINE]

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