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Elucidating the Contribution of Skeletal Muscle Ion Channels to Amyotrophic Lateral Sclerosis in search of new therapeutic options.

Camerino GM, Fonzino A, Conte E, De Bellis M, Mele A, Liantonio A, Tricarico D, Tarantino N, Dobrowolny G, Musarò A, Desaphy JF, De Luca A, Pierno S.

Sci Rep. 2019 Feb 28;9(1):3185. doi: 10.1038/s41598-019-39676-3.


A long-term treatment with taurine prevents cardiac dysfunction in mdx mice.

Mele A, Mantuano P, De Bellis M, Rana F, Sanarica F, Conte E, Morgese MG, Bove M, Rolland JF, Capogrosso RF, Pierno S, Camerino GM, Trabace L, De Luca A.

Transl Res. 2019 Feb;204:82-99. doi: 10.1016/j.trsl.2018.09.004. Epub 2018 Sep 28.


Dual Action of Mexiletine and Its Pyrroline Derivatives as Skeletal Muscle Sodium Channel Blockers and Anti-oxidant Compounds: Toward Novel Therapeutic Potential.

De Bellis M, Sanarica F, Carocci A, Lentini G, Pierno S, Rolland JF, Conte Camerino D, De Luca A.

Front Pharmacol. 2018 Jan 12;8:907. doi: 10.3389/fphar.2017.00907. eCollection 2017.


Ryanodine channel complex stabilizer compound S48168/ARM210 as a disease modifier in dystrophin-deficient mdx mice: proof-of-concept study and independent validation of efficacy.

Capogrosso RF, Mantuano P, Uaesoontrachoon K, Cozzoli A, Giustino A, Dow T, Srinivassane S, Filipovic M, Bell C, Vandermeulen J, Massari AM, De Bellis M, Conte E, Pierno S, Camerino GM, Liantonio A, Nagaraju K, De Luca A.

FASEB J. 2018 Feb;32(2):1025-1043. doi: 10.1096/fj.201700182RRR. Epub 2018 Jan 3.


Risk of Myopathy in Patients in Therapy with Statins: Identification of Biological Markers in a Pilot Study.

Camerino GM, Musumeci O, Conte E, Musaraj K, Fonzino A, Barca E, Marino M, Rodolico C, Tricarico D, Camerino C, Carratù MR, Desaphy JF, De Luca A, Toscano A, Pierno S.

Front Pharmacol. 2017 Jul 27;8:500. doi: 10.3389/fphar.2017.00500. eCollection 2017.


Growth hormone secretagogues prevent dysregulation of skeletal muscle calcium homeostasis in a rat model of cisplatin-induced cachexia.

Conte E, Camerino GM, Mele A, De Bellis M, Pierno S, Rana F, Fonzino A, Caloiero R, Rizzi L, Bresciani E, Ben Haj Salah K, Fehrentz JA, Martinez J, Giustino A, Mariggiò MA, Coluccia M, Tricarico D, Lograno MD, De Luca A, Torsello A, Conte D, Liantonio A.

J Cachexia Sarcopenia Muscle. 2017 Jun;8(3):386-404. doi: 10.1002/jcsm.12185. Epub 2017 Mar 10.


Increased sodium channel use-dependent inhibition by a new potent analogue of tocainide greatly enhances in vivo antimyotonic activity.

De Bellis M, Carbonara R, Roussel J, Farinato A, Massari A, Pierno S, Muraglia M, Corbo F, Franchini C, Carratù MR, De Luca A, Conte Camerino D, Desaphy JF.

Neuropharmacology. 2017 Feb;113(Pt A):206-216. doi: 10.1016/j.neuropharm.2016.10.013. Epub 2016 Oct 13.


Statin-induced myotoxicity is exacerbated by aging: A biophysical and molecular biology study in rats treated with atorvastatin.

Camerino GM, De Bellis M, Conte E, Liantonio A, Musaraj K, Cannone M, Fonzino A, Giustino A, De Luca A, Romano R, Camerino C, Laghezza A, Loiodice F, Desaphy JF, Conte Camerino D, Pierno S.

Toxicol Appl Pharmacol. 2016 Sep 1;306:36-46. doi: 10.1016/j.taap.2016.06.032. Epub 2016 Jul 1.


Therapeutic Approaches to Genetic Ion Channelopathies and Perspectives in Drug Discovery.

Imbrici P, Liantonio A, Camerino GM, De Bellis M, Camerino C, Mele A, Giustino A, Pierno S, De Luca A, Tricarico D, Desaphy JF, Conte D.

Front Pharmacol. 2016 May 10;7:121. doi: 10.3389/fphar.2016.00121. eCollection 2016. Review.


Taurine: the appeal of a safe amino acid for skeletal muscle disorders.

De Luca A, Pierno S, Camerino DC.

J Transl Med. 2015 Jul 25;13:243. doi: 10.1186/s12967-015-0610-1. Review.


Effects of Nandrolone in the Counteraction of Skeletal Muscle Atrophy in a Mouse Model of Muscle Disuse: Molecular Biology and Functional Evaluation.

Camerino GM, Desaphy JF, De Bellis M, Capogrosso RF, Cozzoli A, Dinardo MM, Caloiero R, Musaraj K, Fonzino A, Conte E, Jagerschmidt C, Namour F, Liantonio A, De Luca A, Conte Camerino D, Pierno S.

PLoS One. 2015 Jun 11;10(6):e0129686. doi: 10.1371/journal.pone.0129686. eCollection 2015.


On the metabolically active form of metaglidasen: improved synthesis and investigation of its peculiar activity on peroxisome proliferator-activated receptors and skeletal muscles.

Laghezza A, Montanari R, Lavecchia A, Piemontese L, Pochetti G, Iacobazzi V, Infantino V, Capelli D, De Bellis M, Liantonio A, Pierno S, Tortorella P, Conte Camerino D, Loiodice F.

ChemMedChem. 2015 Mar;10(3):555-65. doi: 10.1002/cmdc.201402462. Epub 2015 Jan 29.


Calcium homeostasis is altered in skeletal muscle of spontaneously hypertensive rats: cytofluorimetric and gene expression analysis.

Liantonio A, Camerino GM, Scaramuzzi A, Cannone M, Pierno S, De Bellis M, Conte E, Fraysse B, Tricarico D, Conte Camerino D.

Am J Pathol. 2014 Oct;184(10):2803-15. doi: 10.1016/j.ajpath.2014.06.020. Epub 2014 Jul 30.


Angiotensin II modulates mouse skeletal muscle resting conductance to chloride and potassium ions and calcium homeostasis via the AT1 receptor and NADPH oxidase.

Cozzoli A, Liantonio A, Conte E, Cannone M, Massari AM, Giustino A, Scaramuzzi A, Pierno S, Mantuano P, Capogrosso RF, Camerino GM, De Luca A.

Am J Physiol Cell Physiol. 2014 Oct 1;307(7):C634-47. doi: 10.1152/ajpcell.00372.2013. Epub 2014 Jul 30.


Protein kinase C theta (PKCθ) modulates the ClC-1 chloride channel activity and skeletal muscle phenotype: a biophysical and gene expression study in mouse models lacking the PKCθ.

Camerino GM, Bouchè M, De Bellis M, Cannone M, Liantonio A, Musaraj K, Romano R, Smeriglio P, Madaro L, Giustino A, De Luca A, Desaphy JF, Camerino DC, Pierno S.

Pflugers Arch. 2014 Dec;466(12):2215-28. doi: 10.1007/s00424-014-1495-1. Epub 2014 Mar 20.


Effects of pleiotrophin overexpression on mouse skeletal muscles in normal loading and in actual and simulated microgravity.

Camerino GM, Pierno S, Liantonio A, De Bellis M, Cannone M, Sblendorio V, Conte E, Mele A, Tricarico D, Tavella S, Ruggiu A, Cancedda R, Ohira Y, Danieli-Betto D, Ciciliot S, Germinario E, Sandonà D, Betto R, Camerino DC, Desaphy JF.

PLoS One. 2013 Aug 28;8(8):e72028. doi: 10.1371/journal.pone.0072028. eCollection 2013.


Growth hormone secretagogues exert differential effects on skeletal muscle calcium homeostasis in male rats depending on the peptidyl/nonpeptidyl structure.

Liantonio A, Gramegna G, Carbonara G, Sblendorio VT, Pierno S, Fraysse B, Giannuzzi V, Rizzi L, Torsello A, Camerino DC.

Endocrinology. 2013 Oct;154(10):3764-75. doi: 10.1210/en.2013-1334. Epub 2013 Jul 8.


Paracrine effects of IGF-1 overexpression on the functional decline due to skeletal muscle disuse: molecular and functional evaluation in hindlimb unloaded MLC/mIgf-1 transgenic mice.

Pierno S, Camerino GM, Cannone M, Liantonio A, De Bellis M, Digennaro C, Gramegna G, De Luca A, Germinario E, Danieli-Betto D, Betto R, Dobrowolny G, Rizzuto E, Musarò A, Desaphy JF, Camerino DC.

PLoS One. 2013 Jun 3;8(6):e65167. doi: 10.1371/journal.pone.0065167. Print 2014.


An olive oil-derived antioxidant mixture ameliorates the age-related decline of skeletal muscle function.

Pierno S, Tricarico D, Liantonio A, Mele A, Digennaro C, Rolland JF, Bianco G, Villanova L, Merendino A, Camerino GM, De Luca A, Desaphy JF, Camerino DC.

Age (Dordr). 2014 Feb;36(1):73-88. doi: 10.1007/s11357-013-9544-9. Epub 2013 May 30.


Potential benefits of taurine in the prevention of skeletal muscle impairment induced by disuse in the hindlimb-unloaded rat.

Pierno S, Liantonio A, Camerino GM, De Bellis M, Cannone M, Gramegna G, Scaramuzzi A, Simonetti S, Nicchia GP, Basco D, Svelto M, Desaphy JF, Camerino DC.

Amino Acids. 2012 Jul;43(1):431-45. doi: 10.1007/s00726-011-1099-4. Epub 2011 Oct 11.


Redox homeostasis, oxidative stress and disuse muscle atrophy.

Pellegrino MA, Desaphy JF, Brocca L, Pierno S, Camerino DC, Bottinelli R.

J Physiol. 2011 May 1;589(Pt 9):2147-60. doi: 10.1113/jphysiol.2010.203232. Epub 2011 Feb 14. Review.


Statin or fibrate chronic treatment modifies the proteomic profile of rat skeletal muscle.

Camerino GM, Pellegrino MA, Brocca L, Digennaro C, Camerino DC, Pierno S, Bottinelli R.

Biochem Pharmacol. 2011 Apr 15;81(8):1054-64. doi: 10.1016/j.bcp.2011.01.022. Epub 2011 Feb 15.


Antioxidant treatment of hindlimb-unloaded mouse counteracts fiber type transition but not atrophy of disused muscles.

Desaphy JF, Pierno S, Liantonio A, Giannuzzi V, Digennaro C, Dinardo MM, Camerino GM, Ricciuti P, Brocca L, Pellegrino MA, Bottinelli R, Camerino DC.

Pharmacol Res. 2010 Jun;61(6):553-63. doi: 10.1016/j.phrs.2010.01.012. Epub 2010 Jan 29.


Is oxidative stress a cause or consequence of disuse muscle atrophy in mice? A proteomic approach in hindlimb-unloaded mice.

Brocca L, Pellegrino MA, Desaphy JF, Pierno S, Camerino DC, Bottinelli R.

Exp Physiol. 2010 Feb;95(2):331-50. doi: 10.1113/expphysiol.2009.050245. Epub 2009 Oct 9.


New 2-aryloxy-3-phenyl-propanoic acids as peroxisome proliferator-activated receptors alpha/gamma dual agonists with improved potency and reduced adverse effects on skeletal muscle function.

Fracchiolla G, Laghezza A, Piemontese L, Tortorella P, Mazza F, Montanari R, Pochetti G, Lavecchia A, Novellino E, Pierno S, Conte Camerino D, Loiodice F.

J Med Chem. 2009 Oct 22;52(20):6382-93. doi: 10.1021/jm900941b.


Statins and fenofibrate affect skeletal muscle chloride conductance in rats by differently impairing ClC-1 channel regulation and expression.

Pierno S, Camerino GM, Cippone V, Rolland JF, Desaphy JF, De Luca A, Liantonio A, Bianco G, Kunic JD, George AL Jr, Conte Camerino D.

Br J Pharmacol. 2009 Apr;156(8):1206-15. doi: 10.1111/j.1476-5381.2008.00079.x. Epub 2009 Feb 13.


Therapeutic approaches to ion channel diseases.

Camerino DC, Desaphy JF, Tricarico D, Pierno S, Liantonio A.

Adv Genet. 2008;64:81-145. doi: 10.1016/S0065-2660(08)00804-3. Review.


Disuse of rat muscle in vivo reduces protein kinase C activity controlling the sarcolemma chloride conductance.

Pierno S, Desaphy JF, Liantonio A, De Luca A, Zarrilli A, Mastrofrancesco L, Procino G, Valenti G, Conte Camerino D.

J Physiol. 2007 Nov 1;584(Pt 3):983-95. Epub 2007 Sep 13.


Role of tumour necrosis factor alpha, but not of cyclo-oxygenase-2-derived eicosanoids, on functional and morphological indices of dystrophic progression in mdx mice: a pharmacological approach.

Pierno S, Nico B, Burdi R, Liantonio A, Didonna MP, Cippone V, Fraysse B, Rolland JF, Mangieri D, Andreetta F, Ferro P, Camerino C, Zallone A, Confalonieri P, De Luca A.

Neuropathol Appl Neurobiol. 2007 Jun;33(3):344-59.


Fluvastatin and atorvastatin affect calcium homeostasis of rat skeletal muscle fibers in vivo and in vitro by impairing the sarcoplasmic reticulum/mitochondria Ca2+-release system.

Liantonio A, Giannuzzi V, Cippone V, Camerino GM, Pierno S, Camerino DC.

J Pharmacol Exp Ther. 2007 May;321(2):626-34. Epub 2007 Feb 9.


Effects of chronic treatment with statins and fenofibrate on rat skeletal muscle: a biochemical, histological and electrophysiological study.

Pierno S, Didonna MP, Cippone V, De Luca A, Pisoni M, Frigeri A, Nicchia GP, Svelto M, Chiesa G, Sirtori C, Scanziani E, Rizzo C, De Vito D, Conte Camerino D.

Br J Pharmacol. 2006 Dec;149(7):909-19. Epub 2006 Oct 9.


Fiber type-related changes in rat skeletal muscle calcium homeostasis during aging and restoration by growth hormone.

Fraysse B, Desaphy JF, Rolland JF, Pierno S, Liantonio A, Giannuzzi V, Camerino C, Didonna MP, Cocchi D, De Luca A, Conte Camerino D.

Neurobiol Dis. 2006 Feb;21(2):372-80. Epub 2005 Sep 8.


Recovery of the soleus muscle after short- and long-term disuse induced by hindlimb unloading: effects on the electrical properties and myosin heavy chain profile.

Desaphy JF, Pierno S, Liantonio A, De Luca A, Didonna MP, Frigeri A, Nicchia GP, Svelto M, Camerino C, Zallone A, Camerino DC.

Neurobiol Dis. 2005 Mar;18(2):356-65.


A multidisciplinary evaluation of the effectiveness of cyclosporine a in dystrophic mdx mice.

De Luca A, Nico B, Liantonio A, Didonna MP, Fraysse B, Pierno S, Burdi R, Mangieri D, Rolland JF, Camerino C, Zallone A, Confalonieri P, Andreetta F, Arnoldi E, Courdier-Fruh I, Magyar JP, Frigeri A, Pisoni M, Svelto M, Conte Camerino D.

Am J Pathol. 2005 Feb;166(2):477-89.


The alteration of calcium homeostasis in adult dystrophic mdx muscle fibers is worsened by a chronic exercise in vivo.

Fraysse B, Liantonio A, Cetrone M, Burdi R, Pierno S, Frigeri A, Pisoni M, Camerino C, De Luca A.

Neurobiol Dis. 2004 Nov;17(2):144-54.


New potent mexiletine and tocainide analogues evaluated in vivo and in vitro as antimyotonic agents on the myotonic ADR mouse.

De Luca A, Pierno S, Liantonio A, Desaphy JF, Natuzzi F, Didonna MP, Ferrannini E, Jockusch H, Franchini C, Lentini G, Corbo F, Tortorella V, Camerino DC.

Neuromuscul Disord. 2004 Jul;14(7):405-16.


Taurine and skeletal muscle disorders.

Conte Camerino D, Tricarico D, Pierno S, Desaphy JF, Liantonio A, Pusch M, Burdi R, Camerino C, Fraysse B, De Luca A.

Neurochem Res. 2004 Jan;29(1):135-42. Review.


Investigations of pharmacologic properties of the renal CLC-K1 chloride channel co-expressed with barttin by the use of 2-(p-Chlorophenoxy)propionic acid derivatives and other structurally unrelated chloride channels blockers.

Liantonio A, Pusch M, Picollo A, Guida P, De Luca A, Pierno S, Fracchiolla G, Loiodice F, Tortorella P, Conte Camerino D.

J Am Soc Nephrol. 2004 Jan;15(1):13-20.


Decrease in resting calcium and calcium entry associated with slow-to-fast transition in unloaded rat soleus muscle.

Fraysse B, Desaphy JF, Pierno S, De Luca A, Liantonio A, Mitolo CI, Camerino DC.

FASEB J. 2003 Oct;17(13):1916-8. Epub 2003 Aug 15.


Structural requisites of 2-(p-chlorophenoxy)propionic acid analogues for activity on native rat skeletal muscle chloride conductance and on heterologously expressed CLC-1.

Liantonio A, De Luca A, Pierno S, Didonna MP, Loiodice F, Fracchiolla G, Tortorella P, Antonio L, Bonerba E, Traverso S, Elia L, Picollo A, Pusch M, Camerino DC.

Br J Pharmacol. 2003 Aug;139(7):1255-64.


Growth hormone secretagogues modulate the electrical and contractile properties of rat skeletal muscle through a ghrelin-specific receptor.

Pierno S, De Luca A, Desaphy JF, Fraysse B, Liantonio A, Didonna MP, Lograno M, Cocchi D, Smith RG, Camerino DC.

Br J Pharmacol. 2003 Jun;139(3):575-84.


Different ability of clenbuterol and salbutamol to block sodium channels predicts their therapeutic use in muscle excitability disorders.

Desaphy JF, Pierno S, De Luca A, Didonna P, Camerino DC.

Mol Pharmacol. 2003 Mar;63(3):659-70.


Enhanced dystrophic progression in mdx mice by exercise and beneficial effects of taurine and insulin-like growth factor-1.

De Luca A, Pierno S, Liantonio A, Cetrone M, Camerino C, Fraysse B, Mirabella M, Servidei S, Rüegg UT, Conte Camerino D.

J Pharmacol Exp Ther. 2003 Jan;304(1):453-63.


Pre-clinical trials in Duchenne dystrophy: what animal models can tell us about potential drug effectiveness.

De Luca A, Pierno S, Liantonio A, Conte Camerino D.

Neuromuscul Disord. 2002 Oct;12 Suppl 1:S142-6. Review.


Molecular requisites for drug binding to muscle CLC-1 and renal CLC-K channel revealed by the use of phenoxy-alkyl derivatives of 2-(p-chlorophenoxy)propionic acid.

Liantonio A, Accardi A, Carbonara G, Fracchiolla G, Loiodice F, Tortorella P, Traverso S, Guida P, Pierno S, De Luca A, Camerino DC, Pusch M.

Mol Pharmacol. 2002 Aug;62(2):265-71.


Change of chloride ion channel conductance is an early event of slow-to-fast fibre type transition during unloading-induced muscle disuse.

Pierno S, Desaphy JF, Liantonio A, De Bellis M, Bianco G, De Luca A, Frigeri A, Nicchia GP, Svelto M, Léoty C, George AL Jr, Camerino DC.

Brain. 2002 Jul;125(Pt 7):1510-21.


Taurine and skeletal muscle ion channels.

De Luca A, Pierno S, Tricarico D, Desaphy JF, Liantonio A, Barbieri M, Camerino C, Montanari L, Camerino DC.

Adv Exp Med Biol. 2000;483:45-56. Review. No abstract available.


Ion channels in muscle and cardiac hereditary diseases: from gene dysfunction to pharmacological therapy. 10-11 March 2000, Bari, Italy.

Desaphy JF, De Luca A, Tricarico D, Pierno S, Conte Camerino D.

Neuromuscul Disord. 2001 Sep;11(6-7):583-8. No abstract available.


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