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. 2001 Aug;7(8):934-40.
doi: 10.1038/90976.

Inter-mitochondrial complementation: Mitochondria-specific system preventing mice from expression of disease phenotypes by mutant mtDNA

K Nakada  1 , K Inoue, T Ono, K Isobe, A Ogura, Y I Goto, I Nonaka, J I Hayashi
Affiliations

Inter-mitochondrial complementation: Mitochondria-specific system preventing mice from expression of disease phenotypes by mutant mtDNA

K Nakada et al. Nat Med. 2001 Aug.

Abstract

Here we investigated the pathogenesis of deletion mutant mitochondrial (mt)DNA by generating mice with mutant mtDNA carrying a 4696-basepair deletion (DeltamtDNA4696), and by using cytochrome c oxidase (COX) electron micrographs to identify COX activity at the individual mitochondrial level. All mitochondria in tissues with DeltamtDNA4696 showed normal COX activity until DeltamtDNA4696 accumulated predominantly; this prevented mice from expressing disease phenotypes. Moreover, we did not observe coexistence of COX-positive and -negative mitochondria within single cells. These results indicate the occurrence of inter-mitochondrial complementation through exchange of genetic contents between exogenously introduced mitochondria with DeltamtDNA4696 and host mitochondria with normal mtDNA. This complementation shows a mitochondria-specific mechanism for avoiding expression of deletion-mutant mtDNA, and opens the possibility of a gene therapy in which mitochondria possessing full-length DNA are introduced.

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Comment in

  • Might mammalian mitochondria merge?
    Turnbull DM, Lightowlers RN. Turnbull DM, et al. Nat Med. 2001 Aug;7(8):895-6. doi: 10.1038/90929. Nat Med. 2001. PMID: 11479619 No abstract available.

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