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Items: 1 to 50 of 311

1.

Evolutionarily conserved susceptibility of the mitochondrial respiratory chain to SDHI pesticides and its consequence on the impact of SDHIs on human cultured cells.

Bénit P, Kahn A, Chretien D, Bortoli S, Huc L, Schiff M, Gimenez-Roqueplo AP, Favier J, Gressens P, Rak M, Rustin P.

PLoS One. 2019 Nov 7;14(11):e0224132. doi: 10.1371/journal.pone.0224132. eCollection 2019.

2.

Arabidopsis thaliana alternative dehydrogenases: a potential therapy for mitochondrial complex I deficiency? Perspectives and pitfalls.

Catania A, Iuso A, Bouchereau J, Kremer LS, Paviolo M, Terrile C, Bénit P, Rasmusson AG, Schwarzmayr T, Tiranti V, Rustin P, Rak M, Prokisch H, Schiff M.

Orphanet J Rare Dis. 2019 Oct 29;14(1):236. doi: 10.1186/s13023-019-1185-3.

3.

Induced pluripotent stem cells-derived neurons from patients with Friedreich ataxia exhibit differential sensitivity to resveratrol and nicotinamide.

Georges P, Boza-Moran MG, Gide J, Pêche GA, Forêt B, Bayot A, Rustin P, Peschanski M, Martinat C, Aubry L.

Sci Rep. 2019 Oct 10;9(1):14568. doi: 10.1038/s41598-019-49870-y.

4.

Report on three additional patients and genotype-phenotype correlation in SLC25A22-related disorders group.

Lemattre C, Imbert-Bouteille M, Gatinois V, Benit P, Sanchez E, Guignard T, Tran Mau-Them F, Haquet E, Rivier F, Carme E, Roubertie A, Boland A, Lechner D, Meyer V, Thevenon J, Duffourd Y, Rivière JB, Deleuze JF, Wells C, Molinari F, Rustin P, Blanchet P, Geneviève D.

Eur J Hum Genet. 2019 Nov;27(11):1692-1700. doi: 10.1038/s41431-019-0433-2. Epub 2019 Jul 8.

PMID:
31285529
5.

Distinctive Krebs cycle remodeling in iPSC-derived neural and mesenchymal stem cells.

Benlamara S, Aubry L, Fabregue J, Bénit P, Rustin P, Rak M.

Biochem Biophys Res Commun. 2019 Apr 9;511(3):658-664. doi: 10.1016/j.bbrc.2019.02.033. Epub 2019 Feb 27.

PMID:
30826061
6.

Lipin1 deficiency causes sarcoplasmic reticulum stress and chaperone-responsive myopathy.

Rashid T, Nemazanyy I, Paolini C, Tatsuta T, Crespin P, de Villeneuve D, Brodesser S, Benit P, Rustin P, Baraibar MA, Agbulut O, Olivier A, Protasi F, Langer T, Chrast R, de Lonlay P, de Foucauld H, Blaauw B, Pende M.

EMBO J. 2019 Jan 3;38(1). pii: e99576. doi: 10.15252/embj.201899576. Epub 2018 Nov 12.

7.

Alternative respiratory chain enzymes: Therapeutic potential and possible pitfalls.

Saari S, Garcia GS, Bremer K, Chioda MM, Andjelković A, Debes PV, Nikinmaa M, Szibor M, Dufour E, Rustin P, Oliveira MT, Jacobs HT.

Biochim Biophys Acta Mol Basis Dis. 2019 Apr 1;1865(4):854-866. doi: 10.1016/j.bbadis.2018.10.012. Epub 2018 Oct 17.

8.

CHCHD2 accumulates in distressed mitochondria and facilitates oligomerization of CHCHD10.

Huang X, Wu BP, Nguyen D, Liu YT, Marani M, Hench J, Bénit P, Kozjak-Pavlovic V, Rustin P, Frank S, Narendra DP.

Hum Mol Genet. 2019 Jan 15;28(2):349. doi: 10.1093/hmg/ddy340. No abstract available.

9.

CHCHD2 accumulates in distressed mitochondria and facilitates oligomerization of CHCHD10.

Huang X, Wu BP, Nguyen D, Liu YT, Marani M, Hench J, Bénit P, Kozjak-Pavlovic V, Rustin P, Frank S, Narendra DP.

Hum Mol Genet. 2018 Nov 15;27(22):3881-3900. doi: 10.1093/hmg/ddy270. Erratum in: Hum Mol Genet. 2019 Jan 15;28(2):349.

PMID:
30084972
10.

ACO2 mutations: A novel phenotype associating severe optic atrophy and spastic paraplegia.

Marelli C, Hamel C, Quiles M, Carlander B, Larrieu L, Delettre C, Sarzi E, Chretien D, Rustin P, Koenig M, Guissart C.

Neurol Genet. 2018 Mar 20;4(2):e225. doi: 10.1212/NXG.0000000000000225. eCollection 2018 Apr. No abstract available.

11.

Mitochondria are physiologically maintained at close to 50 °C.

Chrétien D, Bénit P, Ha HH, Keipert S, El-Khoury R, Chang YT, Jastroch M, Jacobs HT, Rustin P, Rak M.

PLoS Biol. 2018 Jan 25;16(1):e2003992. doi: 10.1371/journal.pbio.2003992. eCollection 2018 Jan.

12.

An Effective, Versatile, and Inexpensive Device for Oxygen Uptake Measurement.

Bénit P, Chrétien D, Porceddu M, Yanicostas C, Rak M, Rustin P.

J Clin Med. 2017 Jun 8;6(6). pii: E58. doi: 10.3390/jcm6060058.

13.

Acute and chronic mitochondrial respiratory chain deficiency differentially regulate lysosomal biogenesis.

Fernández-Mosquera L, Diogo CV, Yambire KF, Santos GL, Luna Sánchez M, Bénit P, Rustin P, Lopez LC, Milosevic I, Raimundo N.

Sci Rep. 2017 Mar 27;7:45076. doi: 10.1038/srep45076.

14.

Paradoxical Inhibition of Glycolysis by Pioglitazone Opposes the Mitochondriopathy Caused by AIF Deficiency.

Bénit P, Pelhaître A, Saunier E, Bortoli S, Coulibaly A, Rak M, Schiff M, Kroemer G, Zeviani M, Rustin P.

EBioMedicine. 2017 Mar;17:75-87. doi: 10.1016/j.ebiom.2017.02.013. Epub 2017 Feb 16.

15.

Broad AOX expression in a genetically tractable mouse model does not disturb normal physiology.

Szibor M, Dhandapani PK, Dufour E, Holmström KM, Zhuang Y, Salwig I, Wittig I, Heidler J, Gizatullina Z, Gainutdinov T; German Mouse Clinic Consortium, Fuchs H, Gailus-Durner V, de Angelis MH, Nandania J, Velagapudi V, Wietelmann A, Rustin P, Gellerich FN, Jacobs HT, Braun T.

Dis Model Mech. 2017 Feb 1;10(2):163-171. doi: 10.1242/dmm.027839. Epub 2016 Dec 14.

16.

QIL1 mutation causes MICOS disassembly and early onset fatal mitochondrial encephalopathy with liver disease.

Guarani V, Jardel C, Chrétien D, Lombès A, Bénit P, Labasse C, Lacène E, Bourillon A, Imbard A, Benoist JF, Dorboz I, Gilleron M, Goetzman ES, Gaignard P, Slama A, Elmaleh-Bergès M, Romero NB, Rustin P, Ogier de Baulny H, Paulo JA, Harper JW, Schiff M.

Elife. 2016 Sep 13;5. pii: e17163. doi: 10.7554/eLife.17163.

17.

The environmental carcinogen benzo[a]pyrene induces a Warburg-like metabolic reprogramming dependent on NHE1 and associated with cell survival.

Hardonnière K, Saunier E, Lemarié A, Fernier M, Gallais I, Héliès-Toussaint C, Mograbi B, Antonio S, Bénit P, Rustin P, Janin M, Habarou F, Ottolenghi C, Lavault MT, Benelli C, Sergent O, Huc L, Bortoli S, Lagadic-Gossmann D.

Sci Rep. 2016 Aug 4;6:30776. doi: 10.1038/srep30776.

18.

[Heart failure from mitochondrial origin: a matter of diet?].

Bénit P, Rustin P.

Med Sci (Paris). 2016 May;32(5):458-60. doi: 10.1051/medsci/20163205011. Epub 2016 May 25. French. No abstract available.

19.

Idebenone in Friedreich ataxia and Leber's hereditary optic neuropathy: close mechanisms, similar therapy?

Schiff M, Rustin P.

Brain. 2016 Jul;139(Pt 7):e39. doi: 10.1093/brain/aww085. Epub 2016 Apr 19. Review. No abstract available.

PMID:
27095078
20.

Expression of the alternative oxidase mitigates beta-amyloid production and toxicity in model systems.

El-Khoury R, Kaulio E, Lassila KA, Crowther DC, Jacobs HT, Rustin P.

Free Radic Biol Med. 2016 Jul;96:57-66. doi: 10.1016/j.freeradbiomed.2016.04.006. Epub 2016 Apr 14.

21.

Translocator Protein-Mediated Stabilization of Mitochondrial Architecture during Inflammation Stress in Colonic Cells.

Issop L, Ostuni MA, Lee S, Laforge M, Péranzi G, Rustin P, Benoist JF, Estaquier J, Papadopoulos V, Lacapère JJ.

PLoS One. 2016 Apr 7;11(4):e0152919. doi: 10.1371/journal.pone.0152919. eCollection 2016.

22.

SLC25A32 Mutations and Riboflavin-Responsive Exercise Intolerance.

Schiff M, Veauville-Merllié A, Su CH, Tzagoloff A, Rak M, Ogier de Baulny H, Boutron A, Smedts-Walters H, Romero NB, Rigal O, Rustin P, Vianey-Saban C, Acquaviva-Bourdain C.

N Engl J Med. 2016 Feb 25;374(8):795-7. doi: 10.1056/NEJMc1513610. No abstract available.

23.

Mitochondrial cytochrome c oxidase deficiency.

Rak M, Bénit P, Chrétien D, Bouchereau J, Schiff M, El-Khoury R, Tzagoloff A, Rustin P.

Clin Sci (Lond). 2016 Mar;130(6):393-407. doi: 10.1042/CS20150707. Review.

24.

Imbalanced OPA1 processing and mitochondrial fragmentation cause heart failure in mice.

Wai T, García-Prieto J, Baker MJ, Merkwirth C, Benit P, Rustin P, Rupérez FJ, Barbas C, Ibañez B, Langer T.

Science. 2015 Dec 4;350(6265):aad0116. doi: 10.1126/science.aad0116.

PMID:
26785494
25.

Diiron centre mutations in Ciona intestinalis alternative oxidase abolish enzymatic activity and prevent rescue of cytochrome oxidase deficiency in flies.

Andjelković A, Oliveira MT, Cannino G, Yalgin C, Dhandapani PK, Dufour E, Rustin P, Szibor M, Jacobs HT.

Sci Rep. 2015 Dec 17;5:18295. doi: 10.1038/srep18295.

26.

Correction: DAPIT Over-Expression Modulates Glucose Metabolism and Cell Behaviour in HEK293T Cells.

Kontro H, Cannino G, Rustin P, Dufour E, Kainulainen H.

PLoS One. 2015 Nov 12;10(11):e0143268. doi: 10.1371/journal.pone.0143268. eCollection 2015. No abstract available.

27.

In Vitro and In Vivo Modulation of Alternative Splicing by the Biguanide Metformin.

Laustriat D, Gide J, Barrault L, Chautard E, Benoit C, Auboeuf D, Boland A, Battail C, Artiguenave F, Deleuze JF, Bénit P, Rustin P, Franc S, Charpentier G, Furling D, Bassez G, Nissan X, Martinat C, Peschanski M, Baghdoyan S.

Mol Ther Nucleic Acids. 2015 Nov 3;4:e262. doi: 10.1038/mtna.2015.35.

28.

In Vivo Detection of Succinate by Magnetic Resonance Spectroscopy as a Hallmark of SDHx Mutations in Paraganglioma.

Lussey-Lepoutre C, Bellucci A, Morin A, Buffet A, Amar L, Janin M, Ottolenghi C, Zinzindohoué F, Autret G, Burnichon N, Robidel E, Banting B, Fontaine S, Cuenod CA, Benit P, Rustin P, Halimi P, Fournier L, Gimenez-Roqueplo AP, Favier J, Tavitian B.

Clin Cancer Res. 2016 Mar 1;22(5):1120-9. doi: 10.1158/1078-0432.CCR-15-1576. Epub 2015 Oct 21.

29.

Correction: DAPIT Over-Expression Modulates Glucose Metabolism and Cell Behaviour in HEK293T Cells.

Kontro H, Cannino G, Rustin P, Dufour E, Kainulainen H.

PLoS One. 2015 Oct 15;10(10):e0141036. doi: 10.1371/journal.pone.0141036. eCollection 2015. No abstract available.

30.

DAPIT Over-Expression Modulates Glucose Metabolism and Cell Behaviour in HEK293T Cells.

Kontro H, Cannino G, Rustin P, Dufour E, Kainulainen H.

PLoS One. 2015 Jul 10;10(7):e0131990. doi: 10.1371/journal.pone.0131990. eCollection 2015. Erratum in: PLoS One. 2015;10(10):e0141036. PLoS One. 2015;10(11):e0143268.

31.

From Nf1 to Sdhb knockout: Successes and failures in the quest for animal models of pheochromocytoma.

Lepoutre-Lussey C, Thibault C, Buffet A, Morin A, Badoual C, Bénit P, Rustin P, Ottolenghi C, Janin M, Castro-Vega LJ, Trapman J, Gimenez-Roqueplo AP, Favier J.

Mol Cell Endocrinol. 2016 Feb 5;421:40-8. doi: 10.1016/j.mce.2015.06.027. Epub 2015 Jun 27. Review.

PMID:
26123588
32.

Interaction between AIF and CHCHD4 Regulates Respiratory Chain Biogenesis.

Hangen E, Féraud O, Lachkar S, Mou H, Doti N, Fimia GM, Lam NV, Zhu C, Godin I, Muller K, Chatzi A, Nuebel E, Ciccosanti F, Flamant S, Bénit P, Perfettini JL, Sauvat A, Bennaceur-Griscelli A, Ser-Le Roux K, Gonin P, Tokatlidis K, Rustin P, Piacentini M, Ruvo M, Blomgren K, Kroemer G, Modjtahedi N.

Mol Cell. 2015 Jun 18;58(6):1001-14. doi: 10.1016/j.molcel.2015.04.020. Epub 2015 May 21.

33.

ANT2-defective fibroblasts exhibit normal mitochondrial bioenergetics.

Prabhu D, Goldstein AC, El-Khoury R, Rak M, Edmunds L, Rustin P, Vockley J, Schiff M.

Mol Genet Metab Rep. 2015 Jun 1;3:43-46.

34.

Genetic and biochemical intricacy shapes mitochondrial cytopathies.

Turnbull DM, Rustin P.

Neurobiol Dis. 2016 Aug;92(Pt A):55-63. doi: 10.1016/j.nbd.2015.02.003. Epub 2015 Feb 12. Review.

PMID:
25684538
35.

Pro-oxidant/antioxidant balance controls pancreatic β-cell differentiation through the ERK1/2 pathway.

Hoarau E, Chandra V, Rustin P, Scharfmann R, Duvillie B.

Cell Death Dis. 2014 Oct 23;5:e1487. doi: 10.1038/cddis.2014.441.

36.

Supernumerary subunits NDUFA3, NDUFA5 and NDUFA12 are required for the formation of the extramembrane arm of human mitochondrial complex I.

Rak M, Rustin P.

FEBS Lett. 2014 May 2;588(9):1832-8. doi: 10.1016/j.febslet.2014.03.046. Epub 2014 Apr 6.

37.

Unsuspected task for an old team: succinate, fumarate and other Krebs cycle acids in metabolic remodeling.

Bénit P, Letouzé E, Rak M, Aubry L, Burnichon N, Favier J, Gimenez-Roqueplo AP, Rustin P.

Biochim Biophys Acta. 2014 Aug;1837(8):1330-7. doi: 10.1016/j.bbabio.2014.03.013. Epub 2014 Mar 31. Review.

38.

Engineering the alternative oxidase gene to better understand and counteract mitochondrial defects: state of the art and perspectives.

El-Khoury R, Kemppainen KK, Dufour E, Szibor M, Jacobs HT, Rustin P.

Br J Pharmacol. 2014 Apr;171(8):2243-9. doi: 10.1111/bph.12570. Review.

39.

Effect of Lon protease knockdown on mitochondrial function in HeLa cells.

Bayot A, Gareil M, Chavatte L, Hamon MP, L'Hermitte-Stead C, Beaumatin F, Priault M, Rustin P, Lombès A, Friguet B, Bulteau AL.

Biochimie. 2014 May;100:38-47. doi: 10.1016/j.biochi.2013.12.005. Epub 2013 Dec 17.

PMID:
24355201
40.

Germline mutations in FH confer predisposition to malignant pheochromocytomas and paragangliomas.

Castro-Vega LJ, Buffet A, De Cubas AA, Cascón A, Menara M, Khalifa E, Amar L, Azriel S, Bourdeau I, Chabre O, Currás-Freixes M, Franco-Vidal V, Guillaud-Bataille M, Simian C, Morin A, Letón R, Gómez-Graña A, Pollard PJ, Rustin P, Robledo M, Favier J, Gimenez-Roqueplo AP.

Hum Mol Genet. 2014 May 1;23(9):2440-6. doi: 10.1093/hmg/ddt639. Epub 2013 Dec 13.

PMID:
24334767
41.

Expression of alternative oxidase in Drosophila ameliorates diverse phenotypes due to cytochrome oxidase deficiency.

Kemppainen KK, Rinne J, Sriram A, Lakanmaa M, Zeb A, Tuomela T, Popplestone A, Singh S, Sanz A, Rustin P, Jacobs HT.

Hum Mol Genet. 2014 Apr 15;23(8):2078-93. doi: 10.1093/hmg/ddt601. Epub 2013 Nov 29.

42.

Friedreich's ataxia, frataxin, PIP5K1B: echo of a distant fracas.

Bayot A, Rustin P.

Oxid Med Cell Longev. 2013;2013:725635. doi: 10.1155/2013/725635. Epub 2013 Sep 30. Review.

43.

Mutations in CYC1, encoding cytochrome c1 subunit of respiratory chain complex III, cause insulin-responsive hyperglycemia.

Gaignard P, Menezes M, Schiff M, Bayot A, Rak M, Ogier de Baulny H, Su CH, Gilleron M, Lombes A, Abida H, Tzagoloff A, Riley L, Cooper ST, Mina K, Sivadorai P, Davis MR, Allcock RJ, Kresoje N, Laing NG, Thorburn DR, Slama A, Christodoulou J, Rustin P.

Am J Hum Genet. 2013 Aug 8;93(2):384-9. doi: 10.1016/j.ajhg.2013.06.015. Epub 2013 Aug 1.

44.

SDH mutations establish a hypermethylator phenotype in paraganglioma.

Letouzé E, Martinelli C, Loriot C, Burnichon N, Abermil N, Ottolenghi C, Janin M, Menara M, Nguyen AT, Benit P, Buffet A, Marcaillou C, Bertherat J, Amar L, Rustin P, De Reyniès A, Gimenez-Roqueplo AP, Favier J.

Cancer Cell. 2013 Jun 10;23(6):739-52. doi: 10.1016/j.ccr.2013.04.018. Epub 2013 May 23.

45.

Disconnecting mitochondrial content from respiratory chain capacity in PGC-1-deficient skeletal muscle.

Rowe GC, Patten IS, Zsengeller ZK, El-Khoury R, Okutsu M, Bampoh S, Koulisis N, Farrell C, Hirshman MF, Yan Z, Goodyear LJ, Rustin P, Arany Z.

Cell Rep. 2013 May 30;3(5):1449-56. doi: 10.1016/j.celrep.2013.04.023. Epub 2013 May 23.

46.

Cis-silencing of PIP5K1B evidenced in Friedreich's ataxia patient cells results in cytoskeleton anomalies.

Bayot A, Reichman S, Lebon S, Csaba Z, Aubry L, Sterkers G, Husson I, Rak M, Rustin P.

Hum Mol Genet. 2013 Jul 15;22(14):2894-904. doi: 10.1093/hmg/ddt144. Epub 2013 Apr 2.

PMID:
23552101
47.

KBP-cytoskeleton interactions underlie developmental anomalies in Goldberg-Shprintzen syndrome.

Drévillon L, Megarbane A, Demeer B, Matar C, Benit P, Briand-Suleau A, Bodereau V, Ghoumid J, Nasser M, Decrouy X, Doco-Fenzy M, Rustin P, Gaillard D, Goossens M, Giurgea I.

Hum Mol Genet. 2013 Jun 15;22(12):2387-99. doi: 10.1093/hmg/ddt083. Epub 2013 Feb 19.

PMID:
23427148
48.

Alternative oxidase expression in the mouse enables bypassing cytochrome c oxidase blockade and limits mitochondrial ROS overproduction.

El-Khoury R, Dufour E, Rak M, Ramanantsoa N, Grandchamp N, Csaba Z, Duvillié B, Bénit P, Gallego J, Gressens P, Sarkis C, Jacobs HT, Rustin P.

PLoS Genet. 2013;9(1):e1003182. doi: 10.1371/journal.pgen.1003182. Epub 2013 Jan 3.

49.

Neuroglobin involvement in respiratory chain function and retinal ganglion cell integrity.

Lechauve C, Augustin S, Cwerman-Thibault H, Bouaita A, Forster V, Célier C, Rustin P, Marden MC, Sahel JA, Corral-Debrinski M.

Biochim Biophys Acta. 2012 Dec;1823(12):2261-73. doi: 10.1016/j.bbamcr.2012.09.009. Epub 2012 Oct 2.

50.

Glucose modulates respiratory complex I activity in response to acute mitochondrial dysfunction.

Cannino G, El-Khoury R, Pirinen M, Hutz B, Rustin P, Jacobs HT, Dufour E.

J Biol Chem. 2012 Nov 9;287(46):38729-40. doi: 10.1074/jbc.M112.386060. Epub 2012 Sep 24.

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