Difference between revisions of "Ahting 2009 Biochim Biophys Acta"
Β |
|||
(9 intermediate revisions by 5 users not shown) | |||
Line 1: | Line 1: | ||
{{Publication | {{Publication | ||
|title=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 | |title=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. | ||
|info=[http://www.ncbi.nlm.nih.gov/pubmed/19111522 PMID: 19111522] | |info=[http://www.ncbi.nlm.nih.gov/pubmed/19111522 PMID: 19111522 Open Access] | ||
|authors=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 | |authors=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 | ||
|year=2009 | |year=2009 | ||
|journal=Biochim | |journal=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. | |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. | ||
|keywords=Tim23 knockout mouse, DDP1,Β Mitochondrial import machinery | |keywords=Tim23 knockout mouse, DDP1,Β Mitochondrial import machinery | ||
|discipline=Mitochondrial Physiology, Biomedicine | |||
}} | }} | ||
{{Labeling | {{Labeling | ||
| | |area=Respiration, Genetic knockout;overexpression | ||
| | |diseases=Other | ||
|organism=Mouse | |organism=Mouse | ||
|preparations= | |tissues=Stem cells | ||
|enzymes=Inner | |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 | |||
}} | }} |
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