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Items: 1 to 50 of 93

1.

Deciphering OPA1 mutations pathogenicity by combined analysis of human, mouse and yeast cell models.

Del Dotto V, Fogazza M, Musiani F, Maresca A, Aleo SJ, Caporali L, La Morgia C, Nolli C, Lodi T, Goffrini P, Chan D, Carelli V, Rugolo M, Baruffini E, Zanna C.

Biochim Biophys Acta Mol Basis Dis. 2018 Oct;1864(10):3496-3514. doi: 10.1016/j.bbadis.2018.08.004. Epub 2018 Aug 4.

PMID:
30293569
2.

Clonal expansion of mtDNA deletions: different disease models assessed by digital droplet PCR in single muscle cells.

Trifunov S, Pyle A, Valentino ML, Liguori R, Yu-Wai-Man P, Burté F, Duff J, Kleinle S, Diebold I, Rugolo M, Horvath R, Carelli V.

Sci Rep. 2018 Aug 3;8(1):11682. doi: 10.1038/s41598-018-30143-z.

3.

Corrigendum to: "Complex II phosphorylation is triggered by unbalanced redox homeostasis in cells lacking complex III." [Biochim. Biophys. Acta 2018;1859(3):182-190.].

Tropeano CV, Fiori J, Carelli V, Caporali L, Daldal F, Ghelli AM, Rugolo M.

Biochim Biophys Acta Bioenerg. 2018 Dec;1859(12):1327. doi: 10.1016/j.bbabio.2018.06.010. Epub 2018 Jun 29. No abstract available.

PMID:
29909117
4.

OPA1: How much do we know to approach therapy?

Del Dotto V, Fogazza M, Lenaers G, Rugolo M, Carelli V, Zanna C.

Pharmacol Res. 2018 May;131:199-210. doi: 10.1016/j.phrs.2018.02.018. Epub 2018 Feb 15. Review.

PMID:
29454676
5.

Eight human OPA1 isoforms, long and short: What are they for?

Del Dotto V, Fogazza M, Carelli V, Rugolo M, Zanna C.

Biochim Biophys Acta Bioenerg. 2018 Apr;1859(4):263-269. doi: 10.1016/j.bbabio.2018.01.005. Epub 2018 Jan 31. Review.

6.

Complex II phosphorylation is triggered by unbalanced redox homeostasis in cells lacking complex III.

Tropeano CV, Fiori J, Carelli V, Caporali L, Daldal F, Ghelli AM, Rugolo M.

Biochim Biophys Acta Bioenerg. 2018 Mar;1859(3):182-190. doi: 10.1016/j.bbabio.2017.12.003. Epub 2017 Dec 18. Erratum in: Biochim Biophys Acta Bioenerg. 2018 Dec;1859(12):1327.

7.

Cellular and mitochondrial determination of low molecular mass organic acids by LC-MS/MS.

Fiori J, Amadesi E, Fanelli F, Tropeano CV, Rugolo M, Gotti R.

J Pharm Biomed Anal. 2018 Feb 20;150:33-38. doi: 10.1016/j.jpba.2017.11.071. Epub 2017 Nov 29.

PMID:
29216582
8.

OPA1 Isoforms in the Hierarchical Organization of Mitochondrial Functions.

Del Dotto V, Mishra P, Vidoni S, Fogazza M, Maresca A, Caporali L, McCaffery JM, Cappelletti M, Baruffini E, Lenaers G, Chan D, Rugolo M, Carelli V, Zanna C.

Cell Rep. 2017 Jun 20;19(12):2557-2571. doi: 10.1016/j.celrep.2017.05.073.

9.

Flammulina velutipes: An option for "alperujo" use.

Rugolo M, Levin L, Lechner BE.

Rev Iberoam Micol. 2016 Oct - Dec;33(4):242-247. doi: 10.1016/j.riam.2015.12.001. Epub 2016 Jul 26.

PMID:
27468641
10.

Syndromic parkinsonism and dementia associated with OPA1 missense mutations.

Carelli V, Musumeci O, Caporali L, Zanna C, La Morgia C, Del Dotto V, Porcelli AM, Rugolo M, Valentino ML, Iommarini L, Maresca A, Barboni P, Carbonelli M, Trombetta C, Valente EM, Patergnani S, Giorgi C, Pinton P, Rizzo G, Tonon C, Lodi R, Avoni P, Liguori R, Baruzzi A, Toscano A, Zeviani M.

Ann Neurol. 2015 Jul;78(1):21-38. doi: 10.1002/ana.24410. Epub 2015 Jun 10.

11.

Mitochondria: Biogenesis and mitophagy balance in segregation and clonal expansion of mitochondrial DNA mutations.

Carelli V, Maresca A, Caporali L, Trifunov S, Zanna C, Rugolo M.

Int J Biochem Cell Biol. 2015 Jun;63:21-4. doi: 10.1016/j.biocel.2015.01.023. Epub 2015 Feb 7. Review.

PMID:
25666555
12.

'Behr syndrome' with OPA1 compound heterozygote mutations.

Carelli V, Sabatelli M, Carrozzo R, Rizza T, Schimpf S, Wissinger B, Zanna C, Rugolo M, La Morgia C, Caporali L, Carbonelli M, Barboni P, Tonon C, Lodi R, Bertini E.

Brain. 2015 Jan;138(Pt 1):e321. doi: 10.1093/brain/awu234. Epub 2014 Aug 21. No abstract available.

13.

A novel in-frame 18-bp microdeletion in MT-CYB causes a multisystem disorder with prominent exercise intolerance.

Carossa V, Ghelli A, Tropeano CV, Valentino ML, Iommarini L, Maresca A, Caporali L, La Morgia C, Liguori R, Barboni P, Carbonelli M, Rizzo G, Tonon C, Lodi R, Martinuzzi A, De Nardo V, Rugolo M, Ferretti L, Gandini F, Pala M, Achilli A, Olivieri A, Torroni A, Carelli V.

Hum Mutat. 2014 Aug;35(8):954-8. doi: 10.1002/humu.22596. Epub 2014 Jun 28.

PMID:
24863938
14.

Respiratory complex I is essential to induce a Warburg profile in mitochondria-defective tumor cells.

Calabrese C, Iommarini L, Kurelac I, Calvaruso MA, Capristo M, Lollini PL, Nanni P, Bergamini C, Nicoletti G, Giovanni CD, Ghelli A, Giorgio V, Caratozzolo MF, Marzano F, Manzari C, Betts CM, Carelli V, Ceccarelli C, Attimonelli M, Romeo G, Fato R, Rugolo M, Tullo A, Gasparre G, Porcelli AM.

Cancer Metab. 2013 Mar 18;1(1):11. doi: 10.1186/2049-3002-1-11.

15.

Different mtDNA mutations modify tumor progression in dependence of the degree of respiratory complex I impairment.

Iommarini L, Kurelac I, Capristo M, Calvaruso MA, Giorgio V, Bergamini C, Ghelli A, Nanni P, De Giovanni C, Carelli V, Fato R, Lollini PL, Rugolo M, Gasparre G, Porcelli AM.

Hum Mol Genet. 2014 Mar 15;23(6):1453-66. doi: 10.1093/hmg/ddt533. Epub 2013 Oct 24.

PMID:
24163135
16.

Gamma rays induce a p53-independent mitochondrial biogenesis that is counter-regulated by HIF1α.

Bartoletti-Stella A, Mariani E, Kurelac I, Maresca A, Caratozzolo MF, Iommarini L, Carelli V, Eusebi LH, Guido A, Cenacchi G, Fuccio L, Rugolo M, Tullo A, Porcelli AM, Gasparre G.

Cell Death Dis. 2013 Jun 13;4:e663. doi: 10.1038/cddis.2013.187.

17.

Molecular mechanisms of superoxide production by complex III: a bacterial versus human mitochondrial comparative case study.

Lanciano P, Khalfaoui-Hassani B, Selamoglu N, Ghelli A, Rugolo M, Daldal F.

Biochim Biophys Acta. 2013 Nov-Dec;1827(11-12):1332-9. doi: 10.1016/j.bbabio.2013.03.009. Epub 2013 Mar 28. Review.

18.

The cytochrome b p.278Y>C mutation causative of a multisystem disorder enhances superoxide production and alters supramolecular interactions of respiratory chain complexes.

Ghelli A, Tropeano CV, Calvaruso MA, Marchesini A, Iommarini L, Porcelli AM, Zanna C, De Nardo V, Martinuzzi A, Wibrand F, Vissing J, Kurelac I, Gasparre G, Selamoglu N, Daldal F, Rugolo M.

Hum Mol Genet. 2013 Jun 1;22(11):2141-51. doi: 10.1093/hmg/ddt067. Epub 2013 Feb 14.

19.

Why mitochondria must fuse to maintain their genome integrity.

Vidoni S, Zanna C, Rugolo M, Sarzi E, Lenaers G.

Antioxid Redox Signal. 2013 Aug 1;19(4):379-88. doi: 10.1089/ars.2012.4800. Epub 2013 Mar 28. Review.

20.

The effects of idebenone on mitochondrial bioenergetics.

Giorgio V, Petronilli V, Ghelli A, Carelli V, Rugolo M, Lenaz G, Bernardi P.

Biochim Biophys Acta. 2012 Feb;1817(2):363-9. doi: 10.1016/j.bbabio.2011.10.012. Epub 2011 Nov 4.

21.

A mutation threshold distinguishes the antitumorigenic effects of the mitochondrial gene MTND1, an oncojanus function.

Gasparre G, Kurelac I, Capristo M, Iommarini L, Ghelli A, Ceccarelli C, Nicoletti G, Nanni P, De Giovanni C, Scotlandi K, Betts CM, Carelli V, Lollini PL, Romeo G, Rugolo M, Porcelli AM.

Cancer Res. 2011 Oct 1;71(19):6220-9. doi: 10.1158/0008-5472.CAN-11-1042. Epub 2011 Aug 18.

22.

A clinically complex form of dominant optic atrophy (OPA8) maps on chromosome 16.

Carelli V, Schimpf S, Fuhrmann N, Valentino ML, Zanna C, Iommarini L, Papke M, Schaich S, Tippmann S, Baumann B, Barboni P, Longanesi L, Rugolo M, Ghelli A, Alavi MV, Youle RJ, Bucchi L, Carroccia R, Giannoccaro MP, Tonon C, Lodi R, Cenacchi G, Montagna P, Liguori R, Wissinger B.

Hum Mol Genet. 2011 May 15;20(10):1893-905. doi: 10.1093/hmg/ddr071. Epub 2011 Feb 24.

PMID:
21349918
23.

OPA1 links human mitochondrial genome maintenance to mtDNA replication and distribution.

Elachouri G, Vidoni S, Zanna C, Pattyn A, Boukhaddaoui H, Gaget K, Yu-Wai-Man P, Gasparre G, Sarzi E, Delettre C, Olichon A, Loiseau D, Reynier P, Chinnery PF, Rotig A, Carelli V, Hamel CP, Rugolo M, Lenaers G.

Genome Res. 2011 Jan;21(1):12-20. doi: 10.1101/gr.108696.110. Epub 2010 Oct 25.

24.

Learning from oncocytic tumors: Why choose inefficient mitochondria?

Gasparre G, Romeo G, Rugolo M, Porcelli AM.

Biochim Biophys Acta. 2011 Jun;1807(6):633-42. doi: 10.1016/j.bbabio.2010.08.006. Epub 2010 Aug 21. Review.

25.

The genetic and metabolic signature of oncocytic transformation implicates HIF1alpha destabilization.

Porcelli AM, Ghelli A, Ceccarelli C, Lang M, Cenacchi G, Capristo M, Pennisi LF, Morra I, Ciccarelli E, Melcarne A, Bartoletti-Stella A, Salfi N, Tallini G, Martinuzzi A, Carelli V, Attimonelli M, Rugolo M, Romeo G, Gasparre G.

Hum Mol Genet. 2010 Mar 15;19(6):1019-32. doi: 10.1093/hmg/ddp566. Epub 2009 Dec 22.

PMID:
20028790
26.

The background of mitochondrial DNA haplogroup J increases the sensitivity of Leber's hereditary optic neuropathy cells to 2,5-hexanedione toxicity.

Ghelli A, Porcelli AM, Zanna C, Vidoni S, Mattioli S, Barbieri A, Iommarini L, Pala M, Achilli A, Torroni A, Rugolo M, Carelli V.

PLoS One. 2009 Nov 19;4(11):e7922. doi: 10.1371/journal.pone.0007922.

27.

An inherited mitochondrial DNA disruptive mutation shifts to homoplasmy in oncocytic tumor cells.

Gasparre G, Iommarini L, Porcelli AM, Lang M, Ferri GG, Kurelac I, Zuntini R, Mariani E, Pennisi LF, Pasquini E, Pasquinelli G, Ghelli A, Bonora E, Ceccarelli C, Rugolo M, Salfi N, Romeo G, Carelli V.

Hum Mutat. 2009 Mar;30(3):391-6. doi: 10.1002/humu.20870.

PMID:
19086058
28.

Respiratory complex I dysfunction due to mitochondrial DNA mutations shifts the voltage threshold for opening of the permeability transition pore toward resting levels.

Porcelli AM, Angelin A, Ghelli A, Mariani E, Martinuzzi A, Carelli V, Petronilli V, Bernardi P, Rugolo M.

J Biol Chem. 2009 Jan 23;284(4):2045-52. doi: 10.1074/jbc.M807321200. Epub 2008 Dec 1.

29.

The antioxidant function of Bcl-2 preserves cytoskeletal stability of cells with defective respiratory complex I.

Porcelli AM, Ghelli A, Iommarini L, Mariani E, Hoque M, Zanna C, Gasparre G, Rugolo M.

Cell Mol Life Sci. 2008 Sep;65(18):2943-51. doi: 10.1007/s00018-008-8300-2.

PMID:
18695940
30.

Protection against oxidant-induced apoptosis by exogenous glutathione in Leber hereditary optic neuropathy cybrids.

Ghelli A, Porcelli AM, Zanna C, Martinuzzi A, Carelli V, Rugolo M.

Invest Ophthalmol Vis Sci. 2008 Feb;49(2):671-6. doi: 10.1167/iovs.07-0880.

PMID:
18235013
31.

OPA1 mutations associated with dominant optic atrophy impair oxidative phosphorylation and mitochondrial fusion.

Zanna C, Ghelli A, Porcelli AM, Karbowski M, Youle RJ, Schimpf S, Wissinger B, Pinti M, Cossarizza A, Vidoni S, Valentino ML, Rugolo M, Carelli V.

Brain. 2008 Feb;131(Pt 2):352-67. doi: 10.1093/brain/awm335.

PMID:
18222991
32.

Rare mtDNA variants in Leber hereditary optic neuropathy families with recurrence of myoclonus.

La Morgia C, Achilli A, Iommarini L, Barboni P, Pala M, Olivieri A, Zanna C, Vidoni S, Tonon C, Lodi R, Vetrugno R, Mostacci B, Liguori R, Carroccia R, Montagna P, Rugolo M, Torroni A, Carelli V.

Neurology. 2008 Mar 4;70(10):762-70. doi: 10.1212/01.wnl.0000295505.74234.d0. Epub 2008 Jan 23.

PMID:
18216301
33.

OPA1 mutations induce mitochondrial DNA instability and optic atrophy 'plus' phenotypes.

Amati-Bonneau P, Valentino ML, Reynier P, Gallardo ME, Bornstein B, Boissière A, Campos Y, Rivera H, de la Aleja JG, Carroccia R, Iommarini L, Labauge P, Figarella-Branger D, Marcorelles P, Furby A, Beauvais K, Letournel F, Liguori R, La Morgia C, Montagna P, Liguori M, Zanna C, Rugolo M, Cossarizza A, Wissinger B, Verny C, Schwarzenbacher R, Martín MA, Arenas J, Ayuso C, Garesse R, Lenaers G, Bonneau D, Carelli V.

Brain. 2008 Feb;131(Pt 2):338-51. Epub 2007 Dec 24.

34.

Green tea modulates alpha(1)-adrenergic stimulated glucose transport in cultured rat cardiomyocytes.

Angeloni C, Maraldi T, Ghelli A, Rugolo M, Leoncini E, Hakim G, Hrelia S.

J Agric Food Chem. 2007 Sep 5;55(18):7553-8. Epub 2007 Aug 4.

PMID:
17676868
35.

Disruptive mitochondrial DNA mutations in complex I subunits are markers of oncocytic phenotype in thyroid tumors.

Gasparre G, Porcelli AM, Bonora E, Pennisi LF, Toller M, Iommarini L, Ghelli A, Moretti M, Betts CM, Martinelli GN, Ceroni AR, Curcio F, Carelli V, Rugolo M, Tallini G, Romeo G.

Proc Natl Acad Sci U S A. 2007 May 22;104(21):9001-6. Epub 2007 May 15.

36.

Defective oxidative phosphorylation in thyroid oncocytic carcinoma is associated with pathogenic mitochondrial DNA mutations affecting complexes I and III.

Bonora E, Porcelli AM, Gasparre G, Biondi A, Ghelli A, Carelli V, Baracca A, Tallini G, Martinuzzi A, Lenaz G, Rugolo M, Romeo G.

Cancer Res. 2006 Jun 15;66(12):6087-96.

37.

Glucose transport activation in human hematopoietic cells M07e is modulated by cytosolic calcium and calmodulin.

Maraldi T, Rugolo M, Fiorentini D, Landi L, Hakim G.

Cell Calcium. 2006 Oct;40(4):373-81. Epub 2006 Jun 9.

PMID:
16762411
38.

Caspase-independent death of Leber's hereditary optic neuropathy cybrids is driven by energetic failure and mediated by AIF and Endonuclease G.

Zanna C, Ghelli A, Porcelli AM, Martinuzzi A, Carelli V, Rugolo M.

Apoptosis. 2005 Oct;10(5):997-1007.

PMID:
16151635
39.

pH difference across the outer mitochondrial membrane measured with a green fluorescent protein mutant.

Porcelli AM, Ghelli A, Zanna C, Pinton P, Rizzuto R, Rugolo M.

Biochem Biophys Res Commun. 2005 Jan 28;326(4):799-804.

PMID:
15607740
40.

Bioenergetics shapes cellular death pathways in Leber's hereditary optic neuropathy: a model of mitochondrial neurodegeneration.

Carelli V, Rugolo M, Sgarbi G, Ghelli A, Zanna C, Baracca A, Lenaz G, Napoli E, Martinuzzi A, Solaini G.

Biochim Biophys Acta. 2004 Jul 23;1658(1-2):172-9. Review.

41.

Staurosporine induces apoptotic volume decrease (AVD) in ECV304 cells.

Porcelli AM, Ghelli A, Zanna C, Valente P, Ferroni S, Rugolo M.

Ann N Y Acad Sci. 2003 Dec;1010:342-6.

PMID:
15033748
42.

Apoptotic cell death of cybrid cells bearing Leber's hereditary optic neuropathy mutations is caspase independent.

Zanna C, Ghelli A, Porcelli AM, Carelli V, Martinuzzi A, Rugolo M.

Ann N Y Acad Sci. 2003 Dec;1010:213-7.

PMID:
15033723
43.

Apoptosis induced by staurosporine in ECV304 cells requires cell shrinkage and upregulation of Cl- conductance.

Porcelli AM, Ghelli A, Zanna C, Valente P, Ferroni S, Rugolo M.

Cell Death Differ. 2004 Jun;11(6):655-62.

44.

Leber's hereditary optic neuropathy (LHON) pathogenic mutations induce mitochondrial-dependent apoptotic death in transmitochondrial cells incubated with galactose medium.

Ghelli A, Zanna C, Porcelli AM, Schapira AH, Martinuzzi A, Carelli V, Rugolo M.

J Biol Chem. 2003 Feb 7;278(6):4145-50. Epub 2002 Nov 21.

45.
47.

Intracellular pH regulation in U-2 OS human osteosarcoma cells transfected with P-glycoprotein.

Porcelli AM, Scotlandi K, Strammiello R, Gislimberti G, Baldini N, Rugolo M.

Biochim Biophys Acta. 2002 Jan 30;1542(1-3):125-38.

48.
49.

Targeting of reporter molecules to mitochondria to measure calcium, ATP, and pH.

Porcelli AM, Pinton P, Ainscow EK, Chiesa A, Rugolo M, Rutter GA, Rizzuto R.

Methods Cell Biol. 2001;65:353-80. No abstract available.

PMID:
11381603
50.

The phorbol ester PMA and cyclic AMP activate different Cl(-) and HCO3(-) fluxes in C127 cells expressing CFTR.

Zegarra-Moran O, Porcelli AM, Rugolo M.

Biochim Biophys Acta. 2001 Feb 14;1535(2):120-7.

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