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Ahting, U. ; Floß, T. ; Uez, N. ; Schneider-Lohmar, I. ; Becker, L. ; Kling, E. ; Iuso, A. ; Bender, A.* ; Hrabě de Angelis, M. ; Gailus-Durner, V. ; Fuchs, H. ; Meitinger, T. ; Wurst, W. ; Prokisch, H. ; Klopstock, T.

Neurological phenotype and reduced lifespan in heterozygous Tim23 knockout mice, the first mouse model of defective mitochondrial import.

Biochim. Biophys. Acta-Bioenerg. 1787, 371-376 (2009)

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The Tim23 protein is the key component of the mitochondrial import machinery. It locates to the inner mitochondrial membrane and its own import is dependent on the DDP1/TIM13 complex. Mutations in human DDP1 cause the Mohr-Tranebjaerg syndrome (MTS/DFN-1; OMIM #304700), which is one of the two known human diseases of the mitochondrial protein import machinery. We created a Tim23 knockout mouse from a gene trap embryonic stem cell clone. Homozygous Tim23 mice were not viable. Heterozygous F1 mutants showed a 50% reduction of Tim23 protein in Western blot, a neurological phenotype and a markedly reduced life span. Haploinsufficiency of the Tim23 mutation underlines the critical role of the mitochondrial import machinery for maintaining mitochondrial function.

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