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Ahting 2009 Biochim Biophys Acta
Coupling states OXPHOS  + , ET  +
Diseases Other  +
Enzyme Inner mt-membrane transporter  + , Complex II;succinate dehydrogenase  + , Complex III  + , Complex IV;cytochrome c oxidase  +
Has abstract The Tim23 protein is the key component of
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.
ry for maintaining mitochondrial function.  +
Has info [http://www.ncbi.nlm.nih.gov/pubmed/19111522 PMID: 19111522 Open Access]  +
Has publicationkeywords Tim23 knockout mouse  + , DDP1  + , Mitochondrial import machinery  +
Has title Ahting U, Floss T, Uez N, Schneider-Lohmar
Ahting U, Floss T, Uez N, Schneider-Lohmar I, Becker L, Kling E, Iuso A, Bender A, de Angelis MH, Gailus-Durner V, Fuchs H, Meitinger T, Wurst W, Prokisch H, Klopstock T (2009) Neurological phenotype and reduced lifespan in heterozygous Tim23 knockout mice, the first mouse model of defective mitochondrial import. Biochim Biophys Acta 1787:371-76.
import. Biochim Biophys Acta 1787:371-76.  +
Instrument and method Oxygraph-2k  +
Mammal and model Mouse  +
MiP area Respiration  + , Genetic knockout;overexpression  +
Pathways N  + , S  +
Preparation Permeabilized cells  +
Tissue and cell Stem cells  +
Was published in journal Biochim Biophys Acta +
Was published in year 2009  +
Was written by Ahting Uwe + , Floss T + , Uez N + , Schneider-Lohmar I + , Becker L + , Kling E + , Iuso Arcangela + , Bender A + , De Angelis MH + , Gailus-Durner V + , Fuchs H + , Meitinger T + , Wurst W + , Prokisch Holger + , Klopstock T +
Categories Publications
Modification date
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09:09:32, 18 October 2018  +
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