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Journal Abstract Search

228 related items for PubMed ID: 25835550

  • 1. Mitochondrial vulnerability and increased susceptibility to nutrient-induced cytotoxicity in fibroblasts from leigh syndrome French canadian patients.
    Burelle Y, Bemeur C, Rivard ME, Thompson Legault J, Boucher G, LSFC Consortium, Morin C, Coderre L, Des Rosiers C.
    PLoS One; 2015; 10(3):e0120767. PubMed ID: 25835550
    [Abstract] [Full Text] [Related]

  • 2. mTORC1 is required for expression of LRPPRC and cytochrome-c oxidase but not HIF-1α in Leigh syndrome French Canadian type patient fibroblasts.
    Mukaneza Y, Cohen A, Rivard MÈ, Tardif J, Deschênes S, Ruiz M, LSFC Consortium, Laprise C, Des Rosiers C, Coderre L.
    Am J Physiol Cell Physiol; 2019 Jul 01; 317(1):C58-C67. PubMed ID: 30995105
    [Abstract] [Full Text] [Related]

  • 3. Low-concentration methylene blue maintains energy production and strongly improves survival of Leigh syndrome French Canadian skin fibroblasts.
    Legault J, Larouche PL, Côté I, Bouchard L, Pichette A, Robinson BH, Morin C.
    J Pharm Pharm Sci; 2011 Jul 01; 14(3):438-49. PubMed ID: 22202226
    [Abstract] [Full Text] [Related]

  • 4. Tissue-specific responses to the LRPPRC founder mutation in French Canadian Leigh Syndrome.
    Sasarman F, Nishimura T, Antonicka H, Weraarpachai W, Shoubridge EA, LSFC Consortium.
    Hum Mol Genet; 2015 Jan 15; 24(2):480-91. PubMed ID: 25214534
    [Abstract] [Full Text] [Related]

  • 5. Expression signature of the Leigh syndrome French-Canadian type.
    Bchetnia M, Tardif J, Morin C, Laprise C.
    Mol Genet Metab Rep; 2022 Mar 15; 30():100847. PubMed ID: 35242578
    [Abstract] [Full Text] [Related]

  • 6. Loss of hepatic LRPPRC alters mitochondrial bioenergetics, regulation of permeability transition and trans-membrane ROS diffusion.
    Cuillerier A, Honarmand S, Cadete VJJ, Ruiz M, Forest A, Deschênes S, Beauchamp C, LSFC Consortium, Charron G, Rioux JD, Des Rosiers C, Shoubridge EA, Burelle Y.
    Hum Mol Genet; 2017 Aug 15; 26(16):3186-3201. PubMed ID: 28575497
    [Abstract] [Full Text] [Related]

  • 7. Novel LRPPRC compound heterozygous mutation in a child with early-onset Leigh syndrome French-Canadian type: case report of an Italian patient.
    Piro E, Serra G, Antona V, Giuffrè M, Giorgio E, Sirchia F, Schierz IAM, Brusco A, Corsello G.
    Ital J Pediatr; 2020 Sep 24; 46(1):140. PubMed ID: 32972427
    [Abstract] [Full Text] [Related]

  • 8. LRPPRC and SLIRP interact in a ribonucleoprotein complex that regulates posttranscriptional gene expression in mitochondria.
    Sasarman F, Brunel-Guitton C, Antonicka H, Wai T, Shoubridge EA, LSFC Consortium.
    Mol Biol Cell; 2010 Apr 15; 21(8):1315-23. PubMed ID: 20200222
    [Abstract] [Full Text] [Related]

  • 9. Lipidomics unveils lipid dyshomeostasis and low circulating plasmalogens as biomarkers in a monogenic mitochondrial disorder.
    Ruiz M, Cuillerier A, Daneault C, Deschênes S, Frayne IR, Bouchard B, Forest A, Legault JT, LSFC Consortium, Vaz FM, Rioux JD, Burelle Y, Des Rosiers C.
    JCI Insight; 2019 Jul 25; 4(14):. PubMed ID: 31341105
    [Abstract] [Full Text] [Related]

  • 10. Effect of thyroid hormone on mitochondrial properties and oxidative stress in cells from patients with mtDNA defects.
    Menzies KJ, Robinson BH, Hood DA.
    Am J Physiol Cell Physiol; 2009 Feb 25; 296(2):C355-62. PubMed ID: 19036942
    [Abstract] [Full Text] [Related]

  • 11. LRPPRC mutations cause early-onset multisystem mitochondrial disease outside of the French-Canadian population.
    Oláhová M, Hardy SA, Hall J, Yarham JW, Haack TB, Wilson WC, Alston CL, He L, Aznauryan E, Brown RM, Brown GK, Morris AA, Mundy H, Broomfield A, Barbosa IA, Simpson MA, Deshpande C, Moeslinger D, Koch J, Stettner GM, Bonnen PE, Prokisch H, Lightowlers RN, McFarland R, Chrzanowska-Lightowlers ZM, Taylor RW.
    Brain; 2015 Dec 25; 138(Pt 12):3503-19. PubMed ID: 26510951
    [Abstract] [Full Text] [Related]

  • 12. Human induced pluripotent stem cells (hiPSCs) derived cells reflect tissue specificity found in patients with Leigh syndrome French Canadian variant (LSFC).
    Gélinas R, Lévesque C, Thompson Legault J, Rivard ME, Villeneuve L, Laprise C, Rioux JD.
    Front Genet; 2024 Dec 25; 15():1375467. PubMed ID: 38706791
    [Abstract] [Full Text] [Related]

  • 13. The role of the LRPPRC (leucine-rich pentatricopeptide repeat cassette) gene in cytochrome oxidase assembly: mutation causes lowered levels of COX (cytochrome c oxidase) I and COX III mRNA.
    Xu F, Morin C, Mitchell G, Ackerley C, Robinson BH.
    Biochem J; 2004 Aug 15; 382(Pt 1):331-6. PubMed ID: 15139850
    [Abstract] [Full Text] [Related]

  • 14. Humoral responses to the measles, mumps and rubella vaccine are impaired in Leigh Syndrome French Canadian patients.
    Fois A, Boucher-Lafleur AM, Thompson Legault J, Renaud C, Morin C, Des Rosiers C, Coderre L, Laprise C, Lesage S, LSFC Consortium.
    PLoS One; 2020 Aug 15; 15(10):e0239860. PubMed ID: 33085679
    [Abstract] [Full Text] [Related]

  • 15. Loss of LRPPRC causes ATP synthase deficiency.
    Mourier A, Ruzzenente B, Brandt T, Kühlbrandt W, Larsson NG.
    Hum Mol Genet; 2014 May 15; 23(10):2580-92. PubMed ID: 24399447
    [Abstract] [Full Text] [Related]

  • 16. Impaired complex IV activity in response to loss of LRPPRC function can be compensated by mitochondrial hyperfusion.
    Rolland SG, Motori E, Memar N, Hench J, Frank S, Winklhofer KF, Conradt B.
    Proc Natl Acad Sci U S A; 2013 Aug 06; 110(32):E2967-76. PubMed ID: 23878239
    [Abstract] [Full Text] [Related]

  • 17. LRPPRC mutations cause a phenotypically distinct form of Leigh syndrome with cytochrome c oxidase deficiency.
    Debray FG, Morin C, Janvier A, Villeneuve J, Maranda B, Laframboise R, Lacroix J, Decarie JC, Robitaille Y, Lambert M, Robinson BH, Mitchell GA.
    J Med Genet; 2011 Mar 06; 48(3):183-9. PubMed ID: 21266382
    [Abstract] [Full Text] [Related]

  • 18. Rescue from galactose-induced death of Leigh Syndrome patient cells by pyruvate and NAD.
    Iannetti EF, Smeitink JAM, Willems PHGM, Beyrath J, Koopman WJH.
    Cell Death Dis; 2018 Nov 14; 9(11):1135. PubMed ID: 30429455
    [Abstract] [Full Text] [Related]

  • 19. Cell-Permeable Succinate Increases Mitochondrial Membrane Potential and Glycolysis in Leigh Syndrome Patient Fibroblasts.
    Bakare AB, Rao RR, Iyer S.
    Cells; 2021 Aug 31; 10(9):. PubMed ID: 34571904
    [Abstract] [Full Text] [Related]

  • 20. Abnormal calcium homeostasis in fibroblasts from patients with Leigh disease.
    Wasniewska M, Karczmarewicz E, Pronicki M, Piekutowska-Abramczuk D, Zablocki K, Popowska E, Pronicka E, Duszyński J.
    Biochem Biophys Res Commun; 2001 May 11; 283(3):687-93. PubMed ID: 11341780
    [Abstract] [Full Text] [Related]

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