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Premature ageing in mice expressing defective mitochondrial DNA polymerase.
Trifunovic A, Wredenberg A, Falkenberg M, Spelbrink JN, Rovio AT, Bruder CE, Bohlooly-Y M, Gidlöf S, Oldfors A, Wibom R, Törnell J, Jacobs HT, Larsson NG. Trifunovic A, et al. Nature. 2004 May 27;429(6990):417-23. doi: 10.1038/nature02517. Nature. 2004. PMID: 15164064
Point mutations and deletions of mitochondrial DNA (mtDNA) accumulate in a variety of tissues during ageing in humans, monkeys and rodents. ...Our results thus provide a causative link between mtDNA mutations and ageing phenotypes in mammals....
Point mutations and deletions of mitochondrial DNA (mtDNA) accumulate in a variety of tissues during ageing in humans, monkeys and ro …
Complex genetic counselling and prenatal analysis in a woman with external ophthalmoplegia and deleted mtDNA.
Graff C, Wredenberg A, Silva JP, Bui TH, Borg K, Larsson NG. Graff C, et al. Prenat Diagn. 2000 May;20(5):426-31. Prenat Diagn. 2000. PMID: 10820414
Single large mitochondrial DNA deletions (DeltamtDNA) are usually spontaneously occurring and cause a wide variety of symptoms, ranging from severe infantile multisystem disorders to adult onset progressive external ophthalmoplegia (PEO). ...This makes it unlikely that the …
Single large mitochondrial DNA deletions (DeltamtDNA) are usually spontaneously occurring and cause a wide variety of symptoms, rangi …
Increased mitochondrial mass in mitochondrial myopathy mice.
Wredenberg A, Wibom R, Wilhelmsson H, Graff C, Wiener HH, Burden SJ, Oldfors A, Westerblad H, Larsson NG. Wredenberg A, et al. Proc Natl Acad Sci U S A. 2002 Nov 12;99(23):15066-71. doi: 10.1073/pnas.232591499. Epub 2002 Nov 4. Proc Natl Acad Sci U S A. 2002. PMID: 12417746 Free PMC article.
We have generated an animal model for mitochondrial myopathy by disrupting the gene for mitochondrial transcription factor A (Tfam) in skeletal muscle of the mouse. ...Enzyme histochemistry, electron micrographs, and citrate synthase activity revealed a substantial …
We have generated an animal model for mitochondrial myopathy by disrupting the gene for mitochondrial transcription factor A (Tfam) i …
Somatic mtDNA mutations cause aging phenotypes without affecting reactive oxygen species production.
Trifunovic A, Hansson A, Wredenberg A, Rovio AT, Dufour E, Khvorostov I, Spelbrink JN, Wibom R, Jacobs HT, Larsson NG. Trifunovic A, et al. Proc Natl Acad Sci U S A. 2005 Dec 13;102(50):17993-8. doi: 10.1073/pnas.0508886102. Epub 2005 Dec 6. Proc Natl Acad Sci U S A. 2005. PMID: 16332961 Free PMC article.
Previously, we reported that mice expressing an error-prone version of the catalytic subunit of mtDNA polymerase accumulate a substantial burden of somatic mtDNA mutations, associated with premature aging phenotypes and reduced lifespan. ...The premature aging phenotypes i …
Previously, we reported that mice expressing an error-prone version of the catalytic subunit of mtDNA polymerase accumulate a substan …
Respiratory chain dysfunction in skeletal muscle does not cause insulin resistance.
Wredenberg A, Freyer C, Sandström ME, Katz A, Wibom R, Westerblad H, Larsson NG. Wredenberg A, et al. Biochem Biophys Res Commun. 2006 Nov 10;350(1):202-7. doi: 10.1016/j.bbrc.2006.09.029. Epub 2006 Sep 18. Biochem Biophys Res Commun. 2006. PMID: 16996481
Insulin resistance in skeletal muscle is a characteristic feature of diabetes mellitus type 2 (DM2). Several lines of circumstantial evidence suggest that reduced mitochondrial oxidative phosphorylation capacity in skeletal muscle is a primary defect causing insulin …
Insulin resistance in skeletal muscle is a characteristic feature of diabetes mellitus type 2 (DM2). Several lines of circumstantial …
Strong purifying selection in transmission of mammalian mitochondrial DNA.
Stewart JB, Freyer C, Elson JL, Wredenberg A, Cansu Z, Trifunovic A, Larsson NG. Stewart JB, et al. PLoS Biol. 2008 Jan;6(1):e10. doi: 10.1371/journal.pbio.0060010. PLoS Biol. 2008. PMID: 18232733 Free PMC article.
We experimentally addressed this issue by studying the maternal transmission of random mtDNA mutations in mtDNA mutator mice expressing a proofreading-deficient mitochondrial DNA polymerase. We report a rapid and strong elimination of nonsynonymous changes in protei …
We experimentally addressed this issue by studying the maternal transmission of random mtDNA mutations in mtDNA mutator mice expressing a
Random point mutations with major effects on protein-coding genes are the driving force behind premature aging in mtDNA mutator mice.
Edgar D, Shabalina I, Camara Y, Wredenberg A, Calvaruso MA, Nijtmans L, Nedergaard J, Cannon B, Larsson NG, Trifunovic A. Edgar D, et al. Cell Metab. 2009 Aug;10(2):131-8. doi: 10.1016/j.cmet.2009.06.010. Cell Metab. 2009. PMID: 19656491
The mtDNA mutator mice have high levels of point mutations and linear deletions of mtDNA causing a progressive respiratory chain dysfunction and a premature aging phenotype. ...Our findings reveal a mechanism for induction of aging phenotypes by demonstrating …
The mtDNA mutator mice have high levels of point mutations and linear deletions of mtDNA causing a progressive respiratory chain dysf …
The bicoid stability factor controls polyadenylation and expression of specific mitochondrial mRNAs in Drosophila melanogaster.
Bratic A, Wredenberg A, Grönke S, Stewart JB, Mourier A, Ruzzenente B, Kukat C, Wibom R, Habermann B, Partridge L, Larsson NG. Bratic A, et al. PLoS Genet. 2011 Oct;7(10):e1002324. doi: 10.1371/journal.pgen.1002324. Epub 2011 Oct 13. PLoS Genet. 2011. PMID: 22022283 Free PMC article.
We have performed immunofluorescence and cell fractionation analyses and show here that BSF is mainly a mitochondrial protein. We studied two independent RNAi knockdown fly lines and report that reduced BSF protein levels lead to a severe respiratory deficiency and …
We have performed immunofluorescence and cell fractionation analyses and show here that BSF is mainly a mitochondrial protein. We stu …
Rescue of primary ubiquinone deficiency due to a novel COQ7 defect using 2,4-dihydroxybensoic acid.
Freyer C, Stranneheim H, Naess K, Mourier A, Felser A, Maffezzini C, Lesko N, Bruhn H, Engvall M, Wibom R, Barbaro M, Hinze Y, Magnusson M, Andeer R, Zetterström RH, von Döbeln U, Wredenberg A, Wedell A. Freyer C, et al. J Med Genet. 2015 Nov;52(11):779-83. doi: 10.1136/jmedgenet-2015-102986. Epub 2015 Jun 17. J Med Genet. 2015. PMID: 26084283 Free PMC article.
Coenzyme Q deficiency has been associated with a range of metabolic diseases, as well as with some drug treatments and ageing. ...CONCLUSION: We report the first patient with primary coenzyme Q deficiency due to a homozygous COQ7 mutation and a potentially be …
Coenzyme Q deficiency has been associated with a range of metabolic diseases, as well as with some drug treatments and ageing. ...CON …
SUV3 helicase is required for correct processing of mitochondrial transcripts.
Clemente P, Pajak A, Laine I, Wibom R, Wedell A, Freyer C, Wredenberg A. Clemente P, et al. Nucleic Acids Res. 2015 Sep 3;43(15):7398-413. doi: 10.1093/nar/gkv692. Epub 2015 Jul 7. Nucleic Acids Res. 2015. PMID: 26152302 Free PMC article.
Furthermore, we observed a severe decrease in mitochondrial tRNAs accompanied by an accumulation of unprocessed precursor transcripts. These processing defects lead to reduced mitochondrial translation and a severe respiratory chain complex deficiency, resulting in …
Furthermore, we observed a severe decrease in mitochondrial tRNAs accompanied by an accumulation of unprocessed precursor transcripts …
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