Article
Neurological phenotype and reduced lifespan in heterozygous Tim23 knockout mice, the first mouse model of defective mitochondrial import.
Biochimica et biophysica acta - 1 May 2009
Ahting Uwe, Floss Thomas, Uez Nikolas, Schneider-Lohmar Ilka, Becker Lore, Kling Eva, Iuso Arcangela, Bender Andreas, de Angelis Martin Hrabé, Gailus-Durner Valérie, Fuchs Helmut, Meitinger Thomas, Wurst Wolfgang, Prokisch Holger, Klopstock Thomas
Abstract excerpt
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...
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