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The mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episode syndrome-associated human mitochondrial tRNALeu(UUR) mutation causes aminoacylation deficiency and concomitant reduced association of mRNA with ribosomes.
Chomyn A, Enriquez JA, Micol V, Fernandez-Silva P, Attardi G. Chomyn A, et al. J Biol Chem. 2000 Jun 23;275(25):19198-209. doi: 10.1074/jbc.M908734199. J Biol Chem. 2000. PMID: 10858457
Cell lines nearly homoplasmic for the mutation exhibited a strong (70-75%) reduction in the level of aminoacylated tRNA(Leu(UUR)) and a decrease in mitochondrial protein synthesis rate. ...Therefore, several lines of evidence indicate that the protein synthesis defe …
Cell lines nearly homoplasmic for the mutation exhibited a strong (70-75%) reduction in the level of aminoacylated tRNA(Leu(UUR)) and …
Proteolytic processing of OPA1 links mitochondrial dysfunction to alterations in mitochondrial morphology.
Duvezin-Caubet S, Jagasia R, Wagener J, Hofmann S, Trifunovic A, Hansson A, Chomyn A, Bauer MF, Attardi G, Larsson NG, Neupert W, Reichert AS. Duvezin-Caubet S, et al. Among authors: chomyn a. J Biol Chem. 2006 Dec 8;281(49):37972-9. doi: 10.1074/jbc.M606059200. Epub 2006 Sep 26. J Biol Chem. 2006. PMID: 17003040 Free article.
MitoTALEN: A General Approach to Reduce Mutant mtDNA Loads and Restore Oxidative Phosphorylation Function in Mitochondrial Diseases.
Hashimoto M, Bacman SR, Peralta S, Falk MJ, Chomyn A, Chan DC, Williams SL, Moraes CT. Hashimoto M, et al. Among authors: chomyn a. Mol Ther. 2015 Oct;23(10):1592-9. doi: 10.1038/mt.2015.126. Epub 2015 Jul 10. Mol Ther. 2015. PMID: 26159306 Free PMC article.
Genetic and functional analysis of mitochondrial DNA-encoded complex I genes.
Bai Y, Hu P, Park JS, Deng JH, Song X, Chomyn A, Yagi T, Attardi G. Bai Y, et al. Among authors: chomyn a. Ann N Y Acad Sci. 2004 Apr;1011:272-83. doi: 10.1007/978-3-662-41088-2_26. Ann N Y Acad Sci. 2004. PMID: 15126303
Mitochondrial fusion is required for mtDNA stability in skeletal muscle and tolerance of mtDNA mutations.
Chen H, Vermulst M, Wang YE, Chomyn A, Prolla TA, McCaffery JM, Chan DC. Chen H, et al. Among authors: chomyn a. Cell. 2010 Apr 16;141(2):280-9. doi: 10.1016/j.cell.2010.02.026. Cell. 2010. PMID: 20403324 Free PMC article.
Aging-related occurrence in Ashkenazi Jews of leukocyte heteroplasmic mtDNA mutation adjacent to replication origin frequently remodeled in Italian centenarians.
Iwata N, Zhang J, Atzmon G, Leanza S, Cho J, Chomyn A, Burk RD, Barzilai N, Attardi G. Iwata N, et al. Among authors: chomyn a. Mitochondrion. 2007 Jul;7(4):267-72. doi: 10.1016/j.mito.2007.03.001. Epub 2007 Mar 12. Mitochondrion. 2007. PMID: 17452024
Disruption of fusion results in mitochondrial heterogeneity and dysfunction.
Chen H, Chomyn A, Chan DC. Chen H, et al. Among authors: chomyn a. J Biol Chem. 2005 Jul 15;280(28):26185-92. doi: 10.1074/jbc.M503062200. Epub 2005 May 17. J Biol Chem. 2005. PMID: 15899901 Free article.
Mitochondrial outer membrane permeability change and hypersensitivity to digitonin early in staurosporine-induced apoptosis.
Duan S, Hajek P, Lin C, Shin SK, Attardi G, Chomyn A. Duan S, et al. J Biol Chem. 2003 Jan 10;278(2):1346-53. doi: 10.1074/jbc.M209269200. Epub 2002 Oct 25. J Biol Chem. 2003. PMID: 12403774
This interpretation was confirmed by another surprising finding, namely that, in staurosporine-treated 143B.TK(-) cells permeabilized with digitonin at a concentration not affecting the mitochondrial membranes in naive cells, the outer mitochondrial membrane loses its inte …
This interpretation was confirmed by another surprising finding, namely that, in staurosporine-treated 143B.TK(-) cells permeabilized with d …
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