Kucharczyk R - Search Results

1

Probing the pathogenicity of patient-derived variants of MT-ATP6 in yeast.

Baranowska E, Niedzwiecka K, Panja C, Charles C, Dautant A, Poznanski J, di Rago JP, Tribouillard-Tanvier D, Kucharczyk R. Baranowska E, et al. Among authors: kucharczyk r. Dis Model Mech. 2023 Apr 1;16(4):dmm049783. doi: 10.1242/dmm.049783. Epub 2023 Apr 21. Dis Model Mech. 2023. PMID: 37083953 Free PMC article.

2

The leader peptide of yeast Atp6p is required for efficient interaction with the Atp9p ring of the mitochondrial ATPase.

Zeng X, Kucharczyk R, di Rago JP, Tzagoloff A. Zeng X, et al. Among authors: kucharczyk r. J Biol Chem. 2007 Dec 14;282(50):36167-76. doi: 10.1074/jbc.M705436200. Epub 2007 Oct 16. J Biol Chem. 2007. PMID: 17940284 Free article.

3

Mitochondrial ATP synthase disorders: molecular mechanisms and the quest for curative therapeutic approaches.

Kucharczyk R, Zick M, Bietenhader M, Rak M, Couplan E, Blondel M, Caubet SD, di Rago JP. Kucharczyk R, et al. Biochim Biophys Acta. 2009 Jan;1793(1):186-99. doi: 10.1016/j.bbamcr.2008.06.012. Epub 2008 Jun 20. Biochim Biophys Acta. 2009. PMID: 18620007 Free article. Review.

4

Biochemical consequences in yeast of the human mitochondrial DNA 8993T>C mutation in the ATPase6 gene found in NARP/MILS patients.

Kucharczyk R, Rak M, di Rago JP. Kucharczyk R, et al. Biochim Biophys Acta. 2009 May;1793(5):817-24. doi: 10.1016/j.bbamcr.2009.02.011. Epub 2009 Mar 6. Biochim Biophys Acta. 2009. PMID: 19269308 Free article.

5

Introducing the human Leigh syndrome mutation T9176G into Saccharomyces cerevisiae mitochondrial DNA leads to severe defects in the incorporation of Atp6p into the ATP synthase and in the mitochondrial morphology.

Kucharczyk R, Salin B, di Rago JP. Kucharczyk R, et al. Hum Mol Genet. 2009 Aug 1;18(15):2889-98. doi: 10.1093/hmg/ddp226. Epub 2009 May 18. Hum Mol Genet. 2009. PMID: 19454486

6

Consequences of the pathogenic T9176C mutation of human mitochondrial DNA on yeast mitochondrial ATP synthase.

Kucharczyk R, Ezkurdia N, Couplan E, Procaccio V, Ackerman SH, Blondel M, di Rago JP. Kucharczyk R, et al. Biochim Biophys Acta. 2010 Jun-Jul;1797(6-7):1105-12. doi: 10.1016/j.bbabio.2009.12.022. Epub 2010 Jan 4. Biochim Biophys Acta. 2010. PMID: 20056103 Free PMC article.

7

A yeast-based assay identifies drugs active against human mitochondrial disorders.

Couplan E, Aiyar RS, Kucharczyk R, Kabala A, Ezkurdia N, Gagneur J, St Onge RP, Salin B, Soubigou F, Le Cann M, Steinmetz LM, di Rago JP, Blondel M. Couplan E, et al. Among authors: kucharczyk r. Proc Natl Acad Sci U S A. 2011 Jul 19;108(29):11989-94. doi: 10.1073/pnas.1101478108. Epub 2011 Jun 29. Proc Natl Acad Sci U S A. 2011. PMID: 21715656 Free PMC article.

8

Defining the pathogenesis of human mtDNA mutations using a yeast model: the case of T8851C.

Kucharczyk R, Giraud MF, Brèthes D, Wysocka-Kapcinska M, Ezkurdia N, Salin B, Velours J, Camougrand N, Haraux F, di Rago JP. Kucharczyk R, et al. Int J Biochem Cell Biol. 2013 Jan;45(1):130-40. doi: 10.1016/j.biocel.2012.07.001. Epub 2012 Jul 10. Int J Biochem Cell Biol. 2013. PMID: 22789932

9

Experimental relocation of the mitochondrial ATP9 gene to the nucleus reveals forces underlying mitochondrial genome evolution.

Bietenhader M, Martos A, Tetaud E, Aiyar RS, Sellem CH, Kucharczyk R, Clauder-Münster S, Giraud MF, Godard F, Salin B, Sagot I, Gagneur J, Déquard-Chablat M, Contamine V, Hermann-Le Denmat S, Sainsard-Chanet A, Steinmetz LM, di Rago JP. Bietenhader M, et al. Among authors: kucharczyk r. PLoS Genet. 2012;8(8):e1002876. doi: 10.1371/journal.pgen.1002876. Epub 2012 Aug 16. PLoS Genet. 2012. PMID: 22916027 Free PMC article.

10

Defining the impact on yeast ATP synthase of two pathogenic human mitochondrial DNA mutations, T9185C and T9191C.

Kabala AM, Lasserre JP, Ackerman SH, di Rago JP, Kucharczyk R. Kabala AM, et al. Among authors: kucharczyk r. Biochimie. 2014 May;100:200-6. doi: 10.1016/j.biochi.2013.11.024. Epub 2013 Dec 4. Biochimie. 2014. PMID: 24316278 Free article.

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