Difference between revisions of "Ahting 2009 Biochim Biophys Acta"
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{{Labeling | {{Labeling | ||
|area=Respiration, Genetic knockout;overexpression | |area=Respiration, Genetic knockout;overexpression | ||
|diseases=Other | |||
|organism=Mouse | |organism=Mouse | ||
|preparations= | |tissues=Stem cells | ||
|enzymes=Inner mt-membrane transporter | |preparations=Permeabilized cells | ||
| | |enzymes=Complex II;succinate dehydrogenase, Complex III, Complex IV;cytochrome c oxidase, Inner mt-membrane transporter | ||
| | |couplingstates=OXPHOS, ET | ||
|pathways=N, S | |||
|instruments=Oxygraph-2k | |instruments=Oxygraph-2k | ||
|discipline=Mitochondrial Physiology, Biomedicine | |discipline=Mitochondrial Physiology, Biomedicine | ||
}} | }} |
Latest revision as of 11:09, 18 October 2018
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. |
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) Biochim Biophys Acta
Abstract: 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. β’ Keywords: Tim23 knockout mouse, DDP1, Mitochondrial import machinery
Labels: MiParea: Respiration, Genetic knockout;overexpression
Pathology: Other
Organism: Mouse Tissue;cell: Stem cells Preparation: Permeabilized cells Enzyme: Complex II;succinate dehydrogenase, Complex III, Complex IV;cytochrome c oxidase, Inner mt-membrane transporter
Coupling state: OXPHOS, ET Pathway: N, S HRR: Oxygraph-2k