Fiorella Piemonte

Fiorella Piemonte

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Fiorella Piemonte

Fiorella Piemonte

Publications by authors named "Fiorella Piemonte"

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Systemic activation of Nrf2 pathway in Parkinson's disease.

Mov Disord 2020 Jan 4;35(1):180-184. Epub 2019 Nov 4.

Neurology, Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.

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http://dx.doi.org/10.1002/mds.27878DOI Listing
January 2020

The NRF2 Signaling Network Defines Clinical Biomarkers and Therapeutic Opportunity in Friedreich's Ataxia.

Int J Mol Sci 2020 01 30;21(3). Epub 2020 Jan 30.

Unit of Muscular and Neurodegenerative Diseases, Bambino Gesù Children's Hospital, IRCCS, 00146 Rome, Italy.

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http://dx.doi.org/10.3390/ijms21030916DOI Listing
January 2020

Targeting NRF2 for the Treatment of Friedreich's Ataxia: A Comparison among Drugs.

Int J Mol Sci 2019 Oct 21;20(20). Epub 2019 Oct 21.

Unit of Muscular and Neurodegenerative Diseases, Bambino Gesù Children's Hospital, IRCCS, 00146 Rome, Italy.

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http://dx.doi.org/10.3390/ijms20205211DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6829337PMC
October 2019

Serum uric acid in Friedreich Ataxia.

Clin Biochem 2018 Apr 2;54:139-141. Epub 2018 Feb 2.

Department of Neurosciences and Neurorehabilitation, IRCCS Bambino Gesù Children Hospital, Rome, Italy.

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https://linkinghub.elsevier.com/retrieve/pii/S00099120173113
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http://dx.doi.org/10.1016/j.clinbiochem.2018.01.022DOI Listing
April 2018

Nrf2-Inducers Counteract Neurodegeneration in Frataxin-Silenced Motor Neurons: Disclosing New Therapeutic Targets for Friedreich's Ataxia.

Int J Mol Sci 2017 Oct 18;18(10). Epub 2017 Oct 18.

Unit of Neuromuscular and Neurodegenerative Diseases, IRCCS Bambino Gesù Children's Hospital, Viale San Paolo 15, 00146 Rome, Italy.

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http://dx.doi.org/10.3390/ijms18102173DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5666854PMC
October 2017

Oxidative stress in Duchenne muscular dystrophy: focus on the NRF2 redox pathway.

Hum Mol Genet 2017 07;26(14):2781-2790

Unit of Muscular and Neurodegenerative Diseases, Children's Hospital and Research Institute Bambino Gesú, 00146 Rome, Italy.

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http://dx.doi.org/10.1093/hmg/ddx173DOI Listing
July 2017

Cytoskeletal dynamics during in vitro neurogenesis of induced pluripotent stem cells (iPSCs).

Mol Cell Neurosci 2016 12 15;77:113-124. Epub 2016 Oct 15.

Department of Neuroscience, Unit of Neuromuscular and Neurodegenerative Diseases, Children's Research Hospital Bambino Gesù, IRCCS, Rome 00146, Italy.

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http://dx.doi.org/10.1016/j.mcn.2016.10.002DOI Listing
December 2016

The cytoskeletal arrangements necessary to neurogenesis.

Oncotarget 2016 Apr;7(15):19414-29

Department of Neuroscience, Unit of Neuromuscular and Neurodegenerative Diseases, Children's Research Hospital Bambino Gesù, Rome, Italy.

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http://dx.doi.org/10.18632/oncotarget.6838DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4991392PMC
April 2016

Systemic Redox Biomarkers in Neurodegenerative Diseases.

Curr Drug Metab 2015 ;16(1):46-70

Unit of Muscular and Neurodegenerative Diseases, Children's Hospital and Research Institute IRCCS Bambino Gesù, Viale di San Paolo 15, 00146, Rome, Italy.

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http://dx.doi.org/10.2174/138920021601150702161250DOI Listing
March 2016

High concentrations of H2O2 trigger hypertrophic cascade and phosphatase and tensin homologue (PTEN) glutathionylation in H9c2 cardiomyocytes.

Exp Mol Pathol 2016 Feb 7;100(1):199-206. Epub 2016 Jan 7.

Liver Research Unit, "Bambino Gesù" Children's Hospital and IRCCS, Viale San Paolo 15, 00146 Rome, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.yexmp.2016.01.001DOI Listing
February 2016

Emodin prevents intrahepatic fat accumulation, inflammation and redox status imbalance during diet-induced hepatosteatosis in rats.

Int J Mol Sci 2012 20;13(2):2276-89. Epub 2012 Feb 20.

Liver Unit of "Bambino Gesù" Children's Hospital, IRCCS, Rome 00165, Italy; E-Mails: (S.C.); (N.P.); (D.G.); (V.N.).

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http://dx.doi.org/10.3390/ijms13022276DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3292022PMC
August 2015

Intracellular distribution of glutathionylated proteins in cultured dermal fibroblasts by immunofluorescence.

Methods Mol Biol 2015 ;1208:395-408

Research Laboratories, Bambino Gesu' Pediatric Hospital IRCCS, P.zza S. Onofrio 4, 00165, Rome, Italy,

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http://dx.doi.org/10.1007/978-1-4939-1441-8_28DOI Listing
June 2015

Friedreich's Ataxia: A Neuronal Point of View on the Oxidative Stress Hypothesis.

Antioxidants (Basel) 2014 Sep 10;3(3):592-603. Epub 2014 Sep 10.

Unit of Neuromuscular and Neurodegenerative diseases, Children's Hospital and Research Institute "Bambino Gesù", 00165 Rome, Italy.

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http://dx.doi.org/10.3390/antiox3030592DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4665420PMC
September 2014

Effects of levosimendan on mitochondrial function in patients with septic shock: a randomized trial.

Biochimie 2014 Jul 19;102:166-73. Epub 2014 Mar 19.

Department of Cardiovascular, Respiratory, Nephrological, Anesthesiological and Geriatric Sciences, University of Rome, "La Sapienza", Italy. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S03009084140008
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http://dx.doi.org/10.1016/j.biochi.2014.03.006DOI Listing
July 2014

Frataxin silencing inactivates mitochondrial Complex I in NSC34 motoneuronal cells and alters glutathione homeostasis.

Int J Mol Sci 2014 Apr 4;15(4):5789-806. Epub 2014 Apr 4.

Unit for Neuromuscular and Neurodegenerative Diseases, Children's Hospital and Research Institute "Bambino Gesù", Piazza S. Onofrio 4, 00165 Rome, Italy.

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http://dx.doi.org/10.3390/ijms15045789DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4013596PMC
April 2014

Glutathione metabolism in cobalamin deficiency type C (cblC).

J Inherit Metab Dis 2014 Jan 9;37(1):125-9. Epub 2013 Apr 9.

Laboratory of Metabolomics and Proteomics, Bambino Gesù Children's Hospital, IRCCS, P.zza S. Onofrio, 4-00165, Rome, Italy,

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http://dx.doi.org/10.1007/s10545-013-9605-3DOI Listing
January 2014

Protein glutathionylation in cardiovascular diseases.

Int J Mol Sci 2013 Oct 17;14(10):20845-76. Epub 2013 Oct 17.

Laboratory of Biochemistry, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

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http://dx.doi.org/10.3390/ijms141020845DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3821647PMC
October 2013

Glutathione imbalance in patients with X-linked adrenoleukodystrophy.

Mol Genet Metab 2013 Aug 22;109(4):366-70. Epub 2013 May 22.

Unit for Neuromuscular and Neurodegenerative Diseases, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

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http://dx.doi.org/10.1016/j.ymgme.2013.05.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3732387PMC
August 2013

Frataxin deficiency leads to reduced expression and impaired translocation of NF-E2-related factor (Nrf2) in cultured motor neurons.

Int J Mol Sci 2013 Apr 10;14(4):7853-65. Epub 2013 Apr 10.

Confocal Microscopy Core Facility, Research Laboratories, Bambino Gesù Children's Hospital, IRCCS, Rome 00165, Italy.

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http://dx.doi.org/10.3390/ijms14047853DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3645720PMC
April 2013

Brown-Vialetto-van Laere and Fazio-Londe overlap syndromes: a clinical, biochemical and genetic study.

Neuromuscul Disord 2012 Dec 21;22(12):1075-82. Epub 2012 Jul 21.

Unit of Neuromuscular Disorders, Rehabilitation and Laboratory of Biochemistry, Bambino Gesu' Children's Research Hospital IRCCS, Rome, Italy.

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https://linkinghub.elsevier.com/retrieve/pii/S09608966120015
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http://dx.doi.org/10.1016/j.nmd.2012.05.007DOI Listing
December 2012

Pediatric reference intervals for muscle coenzyme Q(10).

Biomarkers 2012 Dec 1;17(8):764-6. Epub 2012 Oct 1.

Laboratory of Metabolomics and Proteomics, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy.

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http://dx.doi.org/10.3109/1354750X.2012.727029DOI Listing
December 2012

TMEM70: a mutational hot spot in nuclear ATP synthase deficiency with a pivotal role in complex V biogenesis.

Neurogenetics 2012 Nov 18;13(4):375-86. Epub 2012 Sep 18.

Unit of Molecular Medicine for Neuromuscular and Neurodegenerative Diseases, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

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http://dx.doi.org/10.1007/s10048-012-0343-8DOI Listing
November 2012

S-Glutathionylation signaling in cell biology: progress and prospects.

Eur J Pharm Sci 2012 Aug 30;46(5):279-92. Epub 2012 Mar 30.

Laboratory of Biochemistry, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

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https://linkinghub.elsevier.com/retrieve/pii/S09280987120016
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http://dx.doi.org/10.1016/j.ejps.2012.03.010DOI Listing
August 2012

All glutathione forms are depleted in blood of obese and type 1 diabetic children.

Pediatr Diabetes 2012 May 13;13(3):272-7. Epub 2011 Sep 13.

Laboratory of Biochemistry, Children's Hospital and Research Institute "Bambino Gesù", Rome, Italy.

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http://dx.doi.org/10.1111/j.1399-5448.2011.00806.xDOI Listing
May 2012

Intermittent-relapsing pyruvate dehydrogenase complex deficiency: a case with clinical, biochemical, and neuroradiological reversibility.

Dev Med Child Neurol 2012 May 5;54(5):472-6. Epub 2011 Dec 5.

Department of Neuroscience, Ophthalmology and Genetics, Child Neuropsychiatry Unit, G. Gaslini Institute, and University of Genoa, Genoa, Italy.

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http://dx.doi.org/10.1111/j.1469-8749.2011.04151.xDOI Listing
May 2012

Redox homeostasis and posttranslational modifications/activity of phosphatase and tensin homolog in hepatocytes from rats with diet-induced hepatosteatosis.

J Nutr Biochem 2012 Feb 30;23(2):169-78. Epub 2011 Mar 30.

Unit of Metabolic and Autoimmune Liver Diseases of Bambino Gesù Children's Hospital-IRCCS, Rome, Italy.

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http://dx.doi.org/10.1016/j.jnutbio.2010.11.013DOI Listing
February 2012

Neuroprotection: the emerging concept of restorative neural stem cell biology for the treatment of neurodegenerative diseases.

Curr Neuropharmacol 2011 Jun;9(2):313-7

Unit of Neuromuscular and Neurodegenerative diseases, Children's Hospital and Research Institute "Bambino Gesù", Rome, Italy.

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http://dx.doi.org/10.2174/157015911795596603DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3131722PMC
June 2011

Mirnome analysis reveals novel molecular determinants in the pathogenesis of diet-induced nonalcoholic fatty liver disease.

Lab Invest 2011 Feb 18;91(2):283-93. Epub 2010 Oct 18.

Unit of Metabolic and Autoimmune Liver Diseases, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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http://dx.doi.org/10.1038/labinvest.2010.166DOI Listing
February 2011

Oxidative stress parameters in paediatric non-alcoholic fatty liver disease.

Int J Mol Med 2010 Oct;26(4):471-6

Department of Hepatogastroenterology, Bambino Gesu Children's Hospital, Rome, Italy.

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http://dx.doi.org/10.3892/ijmm_00000487DOI Listing
October 2010

GSSG-mediated Complex I defect in isolated cardiac mitochondria.

Int J Mol Med 2010 Jul;26(1):95-9

Molecular Medicine Unit, Children's Hospital and Research Institute Bambino Gesù, Rome, Italy.

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http://dx.doi.org/10.3892/ijmm_00000439DOI Listing
July 2010

Friedreich's ataxia: oxidative stress and cytoskeletal abnormalities.

J Neurol Sci 2009 Dec 12;287(1-2):111-8. Epub 2009 Sep 12.

Division of Neurology, Department of Neurosciences, Azienda Ospedaliera G Rummo, 82100 Benevento, Italy.

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http://dx.doi.org/10.1016/j.jns.2009.08.052DOI Listing
December 2009

Late-onset MNGIE without peripheral neuropathy due to incomplete loss of thymidine phosphorylase activity.

Neuromuscul Disord 2009 Dec 22;19(12):837-40. Epub 2009 Oct 22.

Department of Neurosciences, University of Rome-Tor Vergata and IRCCS-Fondazione S. Lucia, Via Montpellier 1, I-00135 Rome, Italy.

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http://dx.doi.org/10.1016/j.nmd.2009.08.013DOI Listing
December 2009

Variant late infantile neuronal ceroid lipofuscinosis because of CLN1 mutations.

Pediatr Neurol 2009 Apr;40(4):271-6

Department of Neurological and Visual Sciences, University of Verona School of Medicine, Verona, Italy.

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http://dx.doi.org/10.1016/j.pediatrneurol.2008.10.018DOI Listing
April 2009

Myosin as a potential redox-sensor: an in vitro study.

J Muscle Res Cell Motil 2008 9;29(2-5):119-26. Epub 2008 Sep 9.

Molecular Medicine Unit, Children's Hospital and Research Institute Bambino Gesù, Department of Biology, University of Rome Roma Tre, P.za S. Onofrio, 4, Rome, 00165, Italy.

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http://dx.doi.org/10.1007/s10974-008-9145-xDOI Listing
March 2009

Protein glutathionylation increases in the liver of patients with non-alcoholic fatty liver disease.

J Gastroenterol Hepatol 2008 Aug 6;23(8 Pt 2):e457-64. Epub 2007 Aug 6.

Molecular Medicine Unit, Chemistry Laboratory, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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http://dx.doi.org/10.1111/j.1440-1746.2007.05070.xDOI Listing
August 2008

Collapsing glomerulopathy associated with inherited mitochondrial injury.

Kidney Int 2008 Jul 30;74(2):237-43. Epub 2008 Jan 30.

Department of Pathology and Medicine, School of Medicine, New York University, New York, New York 10016, USA.

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http://dx.doi.org/10.1038/sj.ki.5002767DOI Listing
July 2008

COQ2 nephropathy: a newly described inherited mitochondriopathy with primary renal involvement.

J Am Soc Nephrol 2007 Oct 12;18(10):2773-80. Epub 2007 Sep 12.

Divisions of Pathology , Department of Laboratory Medicine, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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http://dx.doi.org/10.1681/ASN.2006080833DOI Listing
October 2007

Infantile mitochondrial disorders.

Biosci Rep 2007 Jun;27(1-3):105-12

Molecular Medicine, IRCCS Bambino Gesù Hospital, Piazza S. Onofrio 4, 00165, Rome, Italy.

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http://dx.doi.org/10.1007/s10540-007-9039-yDOI Listing
June 2007

Chronic GM2 gangliosidosis type Sandhoff associated with a novel missense HEXB gene mutation causing a double pathogenic effect.

Mol Genet Metab 2007 May 23;91(1):111-4. Epub 2007 Jan 23.

Institute of Neurology, Department of Neuroscience, Catholic University of Sacred Heart, L go A Gemelli 8, Rome, Italy.

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http://dx.doi.org/10.1016/j.ymgme.2006.12.004DOI Listing
May 2007

Leptin, free leptin index, insulin resistance and liver fibrosis in children with non-alcoholic fatty liver disease.

Eur J Endocrinol 2006 Nov;155(5):735-43

Liver Unit, Research Institute, 'Bambino Gesù' Children's Hospital, S Onofrio 4 Square, 00165 Rome, Italy.

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http://dx.doi.org/10.1530/eje.1.02288DOI Listing
November 2006

Novel CLN1 mutation in two Italian sibs with late infantile neuronal ceroid lipofuscinosis.

Eur J Paediatr Neurol 2006 May 8;10(3):154-6. Epub 2006 Jun 8.

Division of Infantile, Neuropsychiatry, Department of Medical and Surgical Pediatrics, University of Messina, via Consolare Valeria 98125 Messina, Italy.

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http://dx.doi.org/10.1016/j.ejpn.2006.04.002DOI Listing
May 2006

Protein glutathionylation in human central nervous system: potential role in redox regulation of neuronal defense against free radicals.

J Neurosci Res 2006 Feb;83(2):256-63

Division of Neurology, Department of Neurosciences, Azienda Ospedaliera G. Rummo, Benevento, Italy.

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http://dx.doi.org/10.1002/jnr.20729DOI Listing
February 2006

Impaired activity of the gamma-glutamyl cycle in nephropathic cystinosis fibroblasts.

Pediatr Res 2006 Feb;59(2):332-5

Department of Nephrology and Urology, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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http://dx.doi.org/10.1203/01.pdr.0000196370.57200.daDOI Listing
February 2006

Simultaneous determination of ubiquinol and ubiquinone in skeletal muscle of pediatric patients.

Anal Biochem 2005 Jul 7;342(2):352-5. Epub 2005 Mar 7.

Laboratory of Biochemistry, Children's Hospital and Research Institute "Bambino Gesù," Piazza S. Onofrio 4, 00165 Rome, Italy.

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http://dx.doi.org/10.1016/j.ab.2005.02.018DOI Listing
July 2005

Glutathione metabolism and antioxidant enzymes in patients affected by nonalcoholic steatohepatitis.

Clin Chim Acta 2005 May;355(1-2):105-11

Division of Hepatology, Children's Hospital and Research Institute "Bambino Gesù," Piazza S. Onofrio, 4, Rome 00165, Italy.

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http://dx.doi.org/10.1016/j.cccn.2004.12.022DOI Listing
May 2005

Actin glutathionylation increases in fibroblasts of patients with Friedreich's ataxia: a potential role in the pathogenesis of the disease.

J Biol Chem 2003 Oct 11;278(43):42588-95. Epub 2003 Aug 11.

Laboratory of Biochemistry, Children's Hospital and Research Institute Bambino Gesù, Piazza S. Onofrio 4, 00165 Rome, Italy.

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http://dx.doi.org/10.1074/jbc.M301872200DOI Listing
October 2003

Analysis of glutathione: implication in redox and detoxification.

Clin Chim Acta 2003 Jul;333(1):19-39

Laboratory of Biochemistry, Children's Hospital and Research Institute Bambino Gesù, Piazza S. Onofrio, 4-00165 Rome, Italy.

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http://dx.doi.org/10.1016/s0009-8981(03)00200-6DOI Listing
July 2003

Glutathione metabolism and antioxidant enzymes in children with Down syndrome.

J Pediatr 2003 May;142(5):583-5

Laboratory of Biochemistry, Molecular Medicine Unit, Children's Hospital and Research Institute Bambino Gesù, Rome, Italy.

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http://dx.doi.org/10.1067/mpd.2003.203DOI Listing
May 2003

Determination of glutathionyl-hemoglobin in human erythrocytes by cation-exchange high-performance liquid chromatography.

Anal Biochem 2003 Jan;312(2):85-90

Laboratory of Biochemistry, Children's Hospital and Research Institute Bambino Gesù, Piazza S. Onofrio, 4, 00165 Rome, Italy.

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http://dx.doi.org/10.1016/s0003-2697(02)00500-6DOI Listing
January 2003

Rapid determination of mycophenolic acid in plasma by reversed-phase high-performance liquid chromatography.

J Chromatogr B Analyt Technol Biomed Life Sci 2002 Sep;776(2):251-4

Laboratory of Clinical Biochemistry, Children's Hospital and Research Institute Bambino Gesù, Viale Oxford, 81 00133 Rome, Italy.

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http://dx.doi.org/10.1016/s1570-0232(02)00353-7DOI Listing
September 2002

Determination of superoxide dismutase and glutathione peroxidase activities in blood of healthy pediatric subjects.

Clin Chim Acta 2002 Aug;322(1-2):117-20

Molecular Medicine Unit, Children's Hospital Bambino Gesù, Piazza S. Onofrio, 4, 00165 Rome, Italy.

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http://dx.doi.org/10.1016/s0009-8981(02)00163-8DOI Listing
August 2002