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Items: 1 to 50 of 105

1.

Venous pressure during intravenous regional anesthesia: Implications for setting tourniquet pressure.

Farhang B, Lesiak AC, Ianno DJ, Minasyan H, Shafritz AB, Viscomi CM.

J Anaesthesiol Clin Pharmacol. 2018 Oct-Dec;34(4):507-512. doi: 10.4103/joacp.JOACP_69_16.

2.

APOPT1/COA8 assists COX assembly and is oppositely regulated by UPS and ROS.

Signes A, Cerutti R, Dickson AS, Benincá C, Hinchy EC, Ghezzi D, Carrozzo R, Bertini E, Murphy MP, Nathan JA, Viscomi C, Fernandez-Vizarra E, Zeviani M.

EMBO Mol Med. 2019 Jan;11(1). pii: e9582. doi: 10.15252/emmm.201809582.

3.

Rapamycin rescues mitochondrial myopathy via coordinated activation of autophagy and lysosomal biogenesis.

Civiletto G, Dogan SA, Cerutti R, Fagiolari G, Moggio M, Lamperti C, Benincá C, Viscomi C, Zeviani M.

EMBO Mol Med. 2018 Nov;10(11). pii: e8799. doi: 10.15252/emmm.201708799.

4.

Towards a therapy for mitochondrial disease: an update.

Garone C, Viscomi C.

Biochem Soc Trans. 2018 Oct 19;46(5):1247-1261. doi: 10.1042/BST20180134. Epub 2018 Oct 8. Review.

5.

Genome editing in mitochondria corrects a pathogenic mtDNA mutation in vivo.

Gammage PA, Viscomi C, Simard ML, Costa ASH, Gaude E, Powell CA, Van Haute L, McCann BJ, Rebelo-Guiomar P, Cerutti R, Zhang L, Rebar EJ, Zeviani M, Frezza C, Stewart JB, Minczuk M.

Nat Med. 2018 Nov;24(11):1691-1695. doi: 10.1038/s41591-018-0165-9. Epub 2018 Sep 24.

PMID:
30250142
6.

Perturbed Redox Signaling Exacerbates a Mitochondrial Myopathy.

Dogan SA, Cerutti R, Benincá C, Brea-Calvo G, Jacobs HT, Zeviani M, Szibor M, Viscomi C.

Cell Metab. 2018 Nov 6;28(5):764-775.e5. doi: 10.1016/j.cmet.2018.07.012. Epub 2018 Aug 16.

7.

Cryo-EM structures of complex I from mouse heart mitochondria in two biochemically defined states.

Agip AA, Blaza JN, Bridges HR, Viscomi C, Rawson S, Muench SP, Hirst J.

Nat Struct Mol Biol. 2018 Jul;25(7):548-556. doi: 10.1038/s41594-018-0073-1. Epub 2018 Jun 18.

8.

Control of mitochondrial superoxide production by reverse electron transport at complex I.

Robb EL, Hall AR, Prime TA, Eaton S, Szibor M, Viscomi C, James AM, Murphy MP.

J Biol Chem. 2018 Jun 22;293(25):9869-9879. doi: 10.1074/jbc.RA118.003647. Epub 2018 May 9.

9.

Lifelong reduction in complex IV induces tissue-specific metabolic effects but does not reduce lifespan or healthspan in mice.

Deepa SS, Pharaoh G, Kinter M, Diaz V, Fok WC, Riddle K, Pulliam D, Hill S, Fischer KE, Soto V, Georgescu C, Wren JD, Viscomi C, Richardson A, Van Remmen H.

Aging Cell. 2018 Apr 25:e12769. doi: 10.1111/acel.12769. [Epub ahead of print]

10.

SURF1 knockout cloned pigs: Early onset of a severe lethal phenotype.

Quadalti C, Brunetti D, Lagutina I, Duchi R, Perota A, Lazzari G, Cerutti R, Di Meo I, Johnson M, Bottani E, Crociara P, Corona C, Grifoni S, Tiranti V, Fernandez-Vizarra E, Robinson AJ, Viscomi C, Casalone C, Zeviani M, Galli C.

Biochim Biophys Acta Mol Basis Dis. 2018 Jun;1864(6 Pt A):2131-2142. doi: 10.1016/j.bbadis.2018.03.021. Epub 2018 Mar 28.

11.

Long-Term Sustained Effect of Liver-Targeted Adeno-Associated Virus Gene Therapy for Mitochondrial Neurogastrointestinal Encephalomyopathy.

Torres-Torronteras J, Cabrera-Pérez R, Vila-Julià F, Viscomi C, Cámara Y, Hirano M, Zeviani M, Martí R.

Hum Gene Ther. 2018 Jun;29(6):708-718. doi: 10.1089/hum.2017.133. Epub 2018 Feb 26.

PMID:
29284302
12.

AAV9-based gene therapy partially ameliorates the clinical phenotype of a mouse model of Leigh syndrome.

Di Meo I, Marchet S, Lamperti C, Zeviani M, Viscomi C.

Gene Ther. 2017 Oct;24(10):661-667. doi: 10.1038/gt.2017.53. Epub 2017 Jul 27.

13.

TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III.

Bottani E, Cerutti R, Harbour ME, Ravaglia S, Dogan SA, Giordano C, Fearnley IM, D'Amati G, Viscomi C, Fernandez-Vizarra E, Zeviani M.

Mol Cell. 2017 Jul 6;67(1):96-105.e4. doi: 10.1016/j.molcel.2017.06.001. Epub 2017 Jun 29.

14.

MtDNA-maintenance defects: syndromes and genes.

Viscomi C, Zeviani M.

J Inherit Metab Dis. 2017 Jul;40(4):587-599. doi: 10.1007/s10545-017-0027-5. Epub 2017 Mar 21. Review.

15.

Down-regulation of the mitochondrial aspartate-glutamate carrier isoform 1 AGC1 inhibits proliferation and N-acetylaspartate synthesis in Neuro2A cells.

Profilo E, Peña-Altamira LE, Corricelli M, Castegna A, Danese A, Agrimi G, Petralla S, Giannuzzi G, Porcelli V, Sbano L, Viscomi C, Massenzio F, Palmieri EM, Giorgi C, Fiermonte G, Virgili M, Palmieri L, Zeviani M, Pinton P, Monti B, Palmieri F, Lasorsa FM.

Biochim Biophys Acta Mol Basis Dis. 2017 Jun;1863(6):1422-1435. doi: 10.1016/j.bbadis.2017.02.022. Epub 2017 Feb 21.

16.

Transcription Factor EB Controls Metabolic Flexibility during Exercise.

Mansueto G, Armani A, Viscomi C, D'Orsi L, De Cegli R, Polishchuk EV, Lamperti C, Di Meo I, Romanello V, Marchet S, Saha PK, Zong H, Blaauw B, Solagna F, Tezze C, Grumati P, Bonaldo P, Pessin JE, Zeviani M, Sandri M, Ballabio A.

Cell Metab. 2017 Jan 10;25(1):182-196. doi: 10.1016/j.cmet.2016.11.003. Epub 2016 Dec 20.

17.

Toward a therapy for mitochondrial disease.

Viscomi C.

Biochem Soc Trans. 2016 Oct 15;44(5):1483-1490. Review.

18.

Effects of ketosis in mitochondrial myopathy: potential benefits of a mitotoxic diet.

Pitceathly RD, Viscomi C.

EMBO Mol Med. 2016 Nov 2;8(11):1231-1233. doi: 10.15252/emmm.201606933. Print 2016 Nov. No abstract available.

19.

FGF21 is a biomarker for mitochondrial translation and mtDNA maintenance disorders.

Lehtonen JM, Forsström S, Bottani E, Viscomi C, Baris OR, Isoniemi H, Höckerstedt K, Österlund P, Hurme M, Jylhävä J, Leppä S, Markkula R, Heliö T, Mombelli G, Uusimaa J, Laaksonen R, Laaksovirta H, Auranen M, Zeviani M, Smeitink J, Wiesner RJ, Nakada K, Isohanni P, Suomalainen A.

Neurology. 2016 Nov 29;87(22):2290-2299. Epub 2016 Oct 28.

20.

Small heterodimer partner 1 directly interacts with NS5A viral protein and has a key role in HCV related liver cell transformation.

Conti B, Porcu C, Viscomi C, Minutolo A, Costantini S, Corazzari M, Iannucci G, Barbaro B, Balsano C.

Oncotarget. 2016 Dec 20;7(51):84575-84586. doi: 10.18632/oncotarget.12144.

21.

Data on cytochrome c oxidase assembly in mice and human fibroblasts or tissues induced by SURF1 defect.

Kovářová N, Pecina P, Nůsková H, Vrbacký M, Zeviani M, Mráček T, Viscomi C, Houštěk J.

Data Brief. 2016 Mar 26;7:1004-9. doi: 10.1016/j.dib.2016.03.065. eCollection 2016 Jun.

22.

Quantitative proteomics suggests metabolic reprogramming during ETHE1 deficiency.

Sahebekhtiari N, Thomsen MM, Sloth JJ, Stenbroen V, Zeviani M, Gregersen N, Viscomi C, Palmfeldt J.

Proteomics. 2016 Apr;16(7):1166-76. doi: 10.1002/pmic.201500336. Epub 2016 Mar 16.

PMID:
26867521
23.

Tissue- and species-specific differences in cytochrome c oxidase assembly induced by SURF1 defects.

Kovářová N, Pecina P, Nůsková H, Vrbacký M, Zeviani M, Mráček T, Viscomi C, Houštěk J.

Biochim Biophys Acta. 2016 Apr;1862(4):705-715. doi: 10.1016/j.bbadis.2016.01.007. Epub 2016 Jan 13.

24.

Defective PITRM1 mitochondrial peptidase is associated with Aβ amyloidotic neurodegeneration.

Brunetti D, Torsvik J, Dallabona C, Teixeira P, Sztromwasser P, Fernandez-Vizarra E, Cerutti R, Reyes A, Preziuso C, D'Amati G, Baruffini E, Goffrini P, Viscomi C, Ferrero I, Boman H, Telstad W, Johansson S, Glaser E, Knappskog PM, Zeviani M, Bindoff LA.

EMBO Mol Med. 2016 Mar 1;8(3):176-90. doi: 10.15252/emmm.201505894.

25.

Core domain mutant Y220C of p53 protein has a key role in copper homeostasis in case of free fatty acids overload.

Arciello M, Longo A, Viscomi C, Capo C, Angeloni A, Rossi L, Balsano C.

Biometals. 2015 Dec;28(6):1017-29. doi: 10.1007/s10534-015-9886-0. Epub 2015 Oct 5.

26.

Reply to Dr Michaud et al.

Elia AE, Viscomi C, La Bella V, Tedeschi G, Albanese A.

Eur J Neurol. 2015 Sep;22(9):e78. doi: 10.1111/ene.12756. No abstract available.

PMID:
26278108
27.

Protective Effect of the Y220C Mutant p53 Against Steatosis: Good News?

Gori M, Barbaro B, Arciello M, Maggio R, Viscomi C, Longo A, Balsano C.

J Cell Physiol. 2015 Jun;230(6):1400. doi: 10.1002/jcp.24874. No abstract available.

PMID:
26179116
28.

Opa1 overexpression ameliorates the phenotype of two mitochondrial disease mouse models.

Civiletto G, Varanita T, Cerutti R, Gorletta T, Barbaro S, Marchet S, Lamperti C, Viscomi C, Scorrano L, Zeviani M.

Cell Metab. 2015 Jun 2;21(6):845-54. doi: 10.1016/j.cmet.2015.04.016.

29.

The OPA1-dependent mitochondrial cristae remodeling pathway controls atrophic, apoptotic, and ischemic tissue damage.

Varanita T, Soriano ME, Romanello V, Zaglia T, Quintana-Cabrera R, Semenzato M, Menabò R, Costa V, Civiletto G, Pesce P, Viscomi C, Zeviani M, Di Lisa F, Mongillo M, Sandri M, Scorrano L.

Cell Metab. 2015 Jun 2;21(6):834-44. doi: 10.1016/j.cmet.2015.05.007.

30.

Emerging concepts in the therapy of mitochondrial disease.

Viscomi C, Bottani E, Zeviani M.

Biochim Biophys Acta. 2015 Jun-Jul;1847(6-7):544-57. doi: 10.1016/j.bbabio.2015.03.001. Epub 2015 Mar 10. Review.

31.

Normocaloric low cholesterol diet modulates Th17/Treg balance in patients with chronic hepatitis C virus infection.

Maggio R, Viscomi C, Andreozzi P, D'Ettorre G, Viscogliosi G, Barbaro B, Gori M, Vullo V, Balsano C.

PLoS One. 2014 Dec 22;9(12):e112346. doi: 10.1371/journal.pone.0112346. eCollection 2014.

32.

Complex IV-deficient Surf1(-/-) mice initiate mitochondrial stress responses.

Pulliam DA, Deepa SS, Liu Y, Hill S, Lin AL, Bhattacharya A, Shi Y, Sloane L, Viscomi C, Zeviani M, Van Remmen H.

Biochem J. 2014 Sep 1;462(2):359-71. doi: 10.1042/BJ20140291.

33.

NAD(+)-dependent activation of Sirt1 corrects the phenotype in a mouse model of mitochondrial disease.

Cerutti R, Pirinen E, Lamperti C, Marchet S, Sauve AA, Li W, Leoni V, Schon EA, Dantzer F, Auwerx J, Viscomi C, Zeviani M.

Cell Metab. 2014 Jun 3;19(6):1042-9. doi: 10.1016/j.cmet.2014.04.001. Epub 2014 May 8.

34.

Pharmacological Inhibition of poly(ADP-ribose) polymerases improves fitness and mitochondrial function in skeletal muscle.

Pirinen E, Cantó C, Jo YS, Morato L, Zhang H, Menzies KJ, Williams EG, Mouchiroud L, Moullan N, Hagberg C, Li W, Timmers S, Imhof R, Verbeek J, Pujol A, van Loon B, Viscomi C, Zeviani M, Schrauwen P, Sauve AA, Schoonjans K, Auwerx J.

Cell Metab. 2014 Jun 3;19(6):1034-41. doi: 10.1016/j.cmet.2014.04.002. Epub 2014 May 8.

35.

Lymphocytes as liver damage mirror of HCV related adipogenesis deregulation.

Minutolo A, Conti B, Grelli S, Viscomi C, Labbadia G, Balsano C.

PLoS One. 2014 Mar 21;9(3):e92343. doi: 10.1371/journal.pone.0092343. eCollection 2014.

36.

Gene therapy using a liver-targeted AAV vector restores nucleoside and nucleotide homeostasis in a murine model of MNGIE.

Torres-Torronteras J, Viscomi C, Cabrera-Pérez R, Cámara Y, Di Meo I, Barquinero J, Auricchio A, Pizzorno G, Hirano M, Zeviani M, Martí R.

Mol Ther. 2014 May;22(5):901-7. doi: 10.1038/mt.2014.6. Epub 2014 Jan 22.

37.

Protective effect of the Y220C mutant p53 against steatosis: good news?

Gori M, Barbaro B, Arciello M, Maggio R, Viscomi C, Longo A, Balsano C.

J Cell Physiol. 2014 Sep;229(9):1182-92. doi: 10.1002/jcp.24550. Erratum in: J Cell Physiol. 2015 Jun;230(6):1400. J Cell Physiol. 2015 Jun;230(6):1400.

PMID:
24395441
38.

AAV-mediated liver-specific MPV17 expression restores mtDNA levels and prevents diet-induced liver failure.

Bottani E, Giordano C, Civiletto G, Di Meo I, Auricchio A, Ciusani E, Marchet S, Lamperti C, d'Amati G, Viscomi C, Zeviani M.

Mol Ther. 2014 Jan;22(1):10-7. doi: 10.1038/mt.2013.230. Epub 2013 Jul 3.

39.

Decreased in vitro mitochondrial function is associated with enhanced brain metabolism, blood flow, and memory in Surf1-deficient mice.

Lin AL, Pulliam DA, Deepa SS, Halloran JJ, Hussong SA, Burbank RR, Bresnen A, Liu Y, Podlutskaya N, Soundararajan A, Muir E, Duong TQ, Bokov AF, Viscomi C, Zeviani M, Richardson AG, Van Remmen H, Fox PT, Galvan V.

J Cereb Blood Flow Metab. 2013 Oct;33(10):1605-11. doi: 10.1038/jcbfm.2013.116. Epub 2013 Jul 10.

40.

Proteome adaptations in Ethe1-deficient mice indicate a role in lipid catabolism and cytoskeleton organization via post-translational protein modifications.

Hildebrandt TM, Di Meo I, Zeviani M, Viscomi C, Braun HP.

Biosci Rep. 2013 Jul 25;33(4). pii: e00052. doi: 10.1042/BSR20130051.

41.

Implementing a fast-track protocol for patients undergoing bowel resection: not so fast.

Hui V, Hyman N, Viscomi C, Osler T.

Am J Surg. 2013 Aug;206(2):152-8. doi: 10.1016/j.amjsurg.2012.11.019. Epub 2013 Jun 4.

PMID:
23759698
42.

Pill counts and pill rental: unintended entrepreneurial opportunities.

Viscomi CM, Covington M, Christenson C.

Clin J Pain. 2013 Jul;29(7):623-4. doi: 10.1097/AJP.0b013e31826f9a24.

PMID:
23328335
43.

Improved insulin sensitivity associated with reduced mitochondrial complex IV assembly and activity.

Deepa SS, Pulliam D, Hill S, Shi Y, Walsh ME, Salmon A, Sloane L, Zhang N, Zeviani M, Viscomi C, Musi N, Van Remmen H.

FASEB J. 2013 Apr;27(4):1371-80. doi: 10.1096/fj.12-221879. Epub 2012 Dec 14.

PMID:
23241310
44.

Effective AAV-mediated gene therapy in a mouse model of ethylmalonic encephalopathy.

Di Meo I, Auricchio A, Lamperti C, Burlina A, Viscomi C, Zeviani M.

EMBO Mol Med. 2012 Sep;4(9):1008-14. doi: 10.1002/emmm.201201433. Epub 2012 Aug 20.

45.

Morphologic evidence of diffuse vascular damage in human and in the experimental model of ethylmalonic encephalopathy.

Giordano C, Viscomi C, Orlandi M, Papoff P, Spalice A, Burlina A, Di Meo I, Tiranti V, Leuzzi V, d'Amati G, Zeviani M.

J Inherit Metab Dis. 2012 May;35(3):451-8. doi: 10.1007/s10545-011-9408-3. Epub 2011 Oct 22.

PMID:
22020834
46.

Clonidine as an adjunct to intravenous regional anesthesia: A randomized, double-blind, placebo-controlled dose ranging study.

Ivie CS, Viscomi CM, Adams DC, Friend AF, Murphy TR, Parker C.

J Anaesthesiol Clin Pharmacol. 2011 Jul;27(3):323-7. doi: 10.4103/0970-9185.83674.

47.

In vivo correction of COX deficiency by activation of the AMPK/PGC-1α axis.

Viscomi C, Bottani E, Civiletto G, Cerutti R, Moggio M, Fagiolari G, Schon EA, Lamperti C, Zeviani M.

Cell Metab. 2011 Jul 6;14(1):80-90. doi: 10.1016/j.cmet.2011.04.011.

48.

Evolution meets disease: penetrance and functional epistasis of mitochondrial tRNA mutations.

Moreno-Loshuertos R, Ferrín G, Acín-Pérez R, Gallardo ME, Viscomi C, Pérez-Martos A, Zeviani M, Fernández-Silva P, Enríquez JA.

PLoS Genet. 2011 Apr;7(4):e1001379. doi: 10.1371/journal.pgen.1001379. Epub 2011 Apr 21.

49.

Deep bradycardia and heart block caused by inducible cardiac-specific knockout of the pacemaker channel gene Hcn4.

Baruscotti M, Bucchi A, Viscomi C, Mandelli G, Consalez G, Gnecchi-Rusconi T, Montano N, Casali KR, Micheloni S, Barbuti A, DiFrancesco D.

Proc Natl Acad Sci U S A. 2011 Jan 25;108(4):1705-10. doi: 10.1073/pnas.1010122108. Epub 2011 Jan 10.

50.

Hypoxic and hypercapnic challenges unveil respiratory vulnerability of Surf1 knockout mice, an animal model of Leigh syndrome.

Stettner GM, Viscomi C, Zeviani M, Wilichowski E, Dutschmann M.

Mitochondrion. 2011 May;11(3):413-20. doi: 10.1016/j.mito.2010.12.011. Epub 2010 Dec 16.

PMID:
21167962

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