Mito-mice: animal models for mitochondrial DNA based diseases

被引：26

作者：

Nakada, K ^{[1
]}

Inoue, K ^{[1
]}

Hayashi, JI ^{[1
]}

机构：

[1] Univ Tsukuba, Inst Biol Sci, Tsukuba, Ibaraki 3058572, Japan

来源：

SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY | 2001年 / 12卷 / 06期

关键词：

mouse model; mitochondrial DNA; large-scale deletion mutation; mitochondrial diseases;

D O I：

10.1006/scdb.2001.0283

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

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 7759-12454 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 rectal 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.

引用

页码：459 / 465

页数：7

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