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Coexistence of CLCN1 and SCN4A mutations in one family suffering from myotonia.

Maggi L, Ravaglia S, Farinato A, Brugnoni R, Altamura C, Imbrici P, Camerino DC, Padovani A, Mantegazza R, Bernasconi P, Desaphy JF, Filosto M.

Neurogenetics. 2017 Dec;18(4):219-225. doi: 10.1007/s10048-017-0525-5. Epub 2017 Oct 9.


Toward precision medicine in myotonic syndromes.

De Bellis M, Camerino DC, Desaphy JF.

Oncotarget. 2017 Feb 28;8(9):14279-14280. doi: 10.18632/oncotarget.15263. No abstract available.


Multidisciplinary study of a new ClC-1 mutation causing myotonia congenita: a paradigm to understand and treat ion channelopathies.

Imbrici P, Altamura C, Camerino GM, Mangiatordi GF, Conte E, Maggi L, Brugnoni R, Musaraj K, Caloiero R, Alberga D, Marsano RM, Ricci G, Siciliano G, Nicolotti O, Mora M, Bernasconi P, Desaphy JF, Mantegazza R, Camerino DC.

FASEB J. 2016 Oct;30(10):3285-3295. Epub 2016 Jun 20.


Kidney CLC-K chloride channels inhibitors: structure-based studies and efficacy in hypertension and associated CLC-K polymorphisms.

Liantonio A, Imbrici P, Camerino GM, Fracchiolla G, Carbonara G, Giannico D, Gradogna A, Mangiatordi GF, Nicolotti O, Tricarico D, Pusch M, Camerino DC.

J Hypertens. 2016 May;34(5):981-92. doi: 10.1097/HJH.0000000000000876.


Inhibition of voltage-gated sodium channels by sumatriptan bioisosteres.

Carbonara R, Carocci A, Roussel J, Crescenzo G, Buonavoglia C, Franchini C, Lentini G, Camerino DC, Desaphy JF.

Front Pharmacol. 2015 Jul 24;6:155. doi: 10.3389/fphar.2015.00155. eCollection 2015.


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.


ClC-1 mutations in myotonia congenita patients: insights into molecular gating mechanisms and genotype-phenotype correlation.

Imbrici P, Maggi L, Mangiatordi GF, Dinardo MM, Altamura C, Brugnoni R, Alberga D, Pinter GL, Ricci G, Siciliano G, Micheli R, Annicchiarico G, Lattanzi G, Nicolotti O, Morandi L, Bernasconi P, Desaphy JF, Mantegazza R, Camerino DC.

J Physiol. 2015 Sep 15;593(18):4181-99. doi: 10.1113/JP270358. Epub 2015 Jul 14.


ClC-1 chloride channels: state-of-the-art research and future challenges.

Imbrici P, Altamura C, Pessia M, Mantegazza R, Desaphy JF, Camerino DC.

Front Cell Neurosci. 2015 Apr 27;9:156. doi: 10.3389/fncel.2015.00156. eCollection 2015. Review.


I-J loop involvement in the pharmacological profile of CLC-K channels expressed in Xenopus oocytes.

Gradogna A, Imbrici P, Zifarelli G, Liantonio A, Camerino DC, Pusch M.

Biochim Biophys Acta. 2014 Nov;1838(11):2745-56. doi: 10.1016/j.bbamem.2014.07.021. Epub 2014 Jul 26.


Actin sliding velocity on pure myosin isoforms from hindlimb unloaded mice.

Maffei M, Longa E, Qaisar R, Agoni V, Desaphy JF, Camerino DC, Bottinelli R, Canepari M.

Acta Physiol (Oxf). 2014 Dec;212(4):316-29. doi: 10.1111/apha.12320. Epub 2014 Jun 30.


Targeting kidney CLC-K channels: pharmacological profile in a human cell line versus Xenopus oocytes.

Imbrici P, Liantonio A, Gradogna A, Pusch M, Camerino DC.

Biochim Biophys Acta. 2014 Oct;1838(10):2484-91. doi: 10.1016/j.bbamem.2014.05.017. Epub 2014 May 24.


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.


Major channels involved in neuropsychiatric disorders and therapeutic perspectives.

Imbrici P, Camerino DC, Tricarico D.

Front Genet. 2013 May 7;4:76. doi: 10.3389/fgene.2013.00076. eCollection 2013.


Combined modifications of mexiletine pharmacophores for new lead blockers of Na(v)1.4 channels.

De Bellis M, De Luca A, Desaphy JF, Carbonara R, Heiny JA, Kennedy A, Carocci A, Cavalluzzi MM, Lentini G, Franchini C, Camerino DC.

Biophys J. 2013 Jan 22;104(2):344-54. doi: 10.1016/j.bpj.2012.11.3830.


Molecular dissection of lubeluzole use-dependent block of voltage-gated sodium channels discloses new therapeutic potentials.

Desaphy JF, Carbonara R, Costanza T, Lentini G, Cavalluzzi MM, Bruno C, Franchini C, Camerino DC.

Mol Pharmacol. 2013 Feb;83(2):406-15. doi: 10.1124/mol.112.080804. Epub 2012 Nov 21.


Dramatic improvement of myotonia permanens with flecainide: a two-case report of a possible bench-to-bedside pharmacogenetics strategy.

Desaphy JF, Modoni A, Lomonaco M, Camerino DC.

Eur J Clin Pharmacol. 2013 Apr;69(4):1037-9. doi: 10.1007/s00228-012-1414-3. Epub 2012 Oct 3. No abstract available.


Opening/blocking actions of pyruvate kinase antibodies on neuronal and muscular KATP channels.

Mele A, Buttiglione M, Cannone G, Vitiello F, Camerino DC, Tricarico D.

Pharmacol Res. 2012 Nov;66(5):401-8. doi: 10.1016/j.phrs.2012.07.007. Epub 2012 Aug 9.


Molecular Insights into the Local Anesthetic Receptor within Voltage-Gated Sodium Channels Using Hydroxylated Analogs of Mexiletine.

Desaphy JF, Dipalma A, Costanza T, Carbonara R, Dinardo MM, Catalano A, Carocci A, Lentini G, Franchini C, Camerino DC.

Front Pharmacol. 2012 Feb 15;3:17. doi: 10.3389/fphar.2012.00017. eCollection 2012.


In-vivo administration of CLC-K kidney chloride channels inhibitors increases water diuresis in rats: a new drug target for hypertension?

Liantonio A, Gramegna G, Camerino GM, Dinardo MM, Scaramuzzi A, Potenza MA, Montagnani M, Procino G, Lasorsa DR, Mastrofrancesco L, Laghezza A, Fracchiolla G, Loiodice F, Perrone MG, Lopedota A, Conte S, Penza R, Valenti G, Svelto M, Camerino DC.

J Hypertens. 2012 Jan;30(1):153-67. doi: 10.1097/HJH.0b013e32834d9eb9.


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.


Searching for novel anti-myotonic agents: pharmacophore requirement for use-dependent block of skeletal muscle sodium channels by N-benzylated cyclic derivatives of tocainide.

De Luca A, De Bellis M, Corbo F, Franchini C, Muraglia M, Catalano A, Carocci A, Camerino DC.

Neuromuscul Disord. 2012 Jan;22(1):56-65. doi: 10.1016/j.nmd.2011.07.001. Epub 2011 Jul 29.


Recent advances in the pathogenesis and drug action in periodic paralyses and related channelopathies.

Tricarico D, Camerino DC.

Front Pharmacol. 2011 Feb 28;2:8. doi: 10.3389/fphar.2011.00008. eCollection 2011.


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.


Analysis by two-dimensional Blue Native/SDS-PAGE of membrane protein alterations in rat soleus muscle after hindlimb unloading.

Basco D, Nicchia GP, Desaphy JF, Camerino DC, Frigeri A, Svelto M.

Eur J Appl Physiol. 2010 Dec;110(6):1215-24. doi: 10.1007/s00421-010-1592-6. Epub 2010 Aug 24.


Identification of sites responsible for the potentiating effect of niflumic acid on ClC-Ka kidney chloride channels.

Zifarelli G, Liantonio A, Gradogna A, Picollo A, Gramegna G, De Bellis M, Murgia AR, Babini E, Camerino DC, Pusch M.

Br J Pharmacol. 2010 Aug;160(7):1652-61. doi: 10.1111/j.1476-5381.2010.00822.x.


Effects of a new potent analog of tocainide on hNav1.7 sodium channels and in vivo neuropathic pain models.

Ghelardini C, Desaphy JF, Muraglia M, Corbo F, Matucci R, Dipalma A, Bertucci C, Pistolozzi M, Nesi M, Norcini M, Franchini C, Camerino DC.

Neuroscience. 2010 Aug 25;169(2):863-73. doi: 10.1016/j.neuroscience.2010.05.019. Epub 2010 May 16.


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.


The KATP channel is a molecular sensor of atrophy in skeletal muscle.

Tricarico D, Mele A, Camerino GM, Bottinelli R, Brocca L, Frigeri A, Svelto M, George AL Jr, Camerino DC.

J Physiol. 2010 Mar 1;588(Pt 5):773-84. doi: 10.1113/jphysiol.2009.185835. Epub 2010 Jan 11.


Grand challenge for ion channels: an underexploited resource for therapeutics.

Camerino DC, Desaphy JF.

Front Pharmacol. 2010 Aug 20;1:113. doi: 10.3389/fphar.2010.00113. eCollection 2010. No abstract available.


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.


Involvement of voltage-gated sodium channels blockade in the analgesic effects of orphenadrine.

Desaphy JF, Dipalma A, De Bellis M, Costanza T, Gaudioso C, Delmas P, George AL Jr, Camerino DC.

Pain. 2009 Apr;142(3):225-35. doi: 10.1016/j.pain.2009.01.010. Epub 2009 Feb 12.


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.


Effect of microgravity on gene expression in mouse brain.

Frigeri A, Iacobas DA, Iacobas S, Nicchia GP, Desaphy JF, Camerino DC, Svelto M, Spray DC.

Exp Brain Res. 2008 Nov;191(3):289-300. doi: 10.1007/s00221-008-1523-5. Epub 2008 Aug 14.


Gentamicin treatment in exercised mdx mice: Identification of dystrophin-sensitive pathways and evaluation of efficacy in work-loaded dystrophic muscle.

De Luca A, Nico B, Rolland JF, Cozzoli A, Burdi R, Mangieri D, Giannuzzi V, Liantonio A, Cippone V, De Bellis M, Nicchia GP, Camerino GM, Frigeri A, Svelto M, Camerino DC.

Neurobiol Dis. 2008 Nov;32(2):243-53. doi: 10.1016/j.nbd.2008.07.009. Epub 2008 Jul 23.


Molecular determinants for the activating/blocking actions of the 2H-1,4-benzoxazine derivatives, a class of potassium channel modulators targeting the skeletal muscle KATP channels.

Tricarico D, Mele A, Camerino GM, Laghezza A, Carbonara G, Fracchiolla G, Tortorella P, Loiodice F, Camerino DC.

Mol Pharmacol. 2008 Jul;74(1):50-8. doi: 10.1124/mol.108.046615. Epub 2008 Apr 10.


Acetazolamide prevents vacuolar myopathy in skeletal muscle of K(+) -depleted rats.

Tricarico D, Lovaglio S, Mele A, Rotondo G, Mancinelli E, Meola G, Camerino DC.

Br J Pharmacol. 2008 May;154(1):183-90. doi: 10.1038/bjp.2008.42. Epub 2008 Mar 17.


Constrained analogues of tocainide as potent skeletal muscle sodium channel blockers towards the development of antimyotonic agents.

Catalano A, Carocci A, Corbo F, Franchini C, Muraglia M, Scilimati A, De Bellis M, De Luca A, Camerino DC, Sinicropi MS, Tortorella V.

Eur J Med Chem. 2008 Nov;43(11):2535-40. doi: 10.1016/j.ejmech.2008.01.023. Epub 2008 Jan 31.


Molecular switch for CLC-K Cl- channel block/activation: optimal pharmacophoric requirements towards high-affinity ligands.

Liantonio A, Picollo A, Carbonara G, Fracchiolla G, Tortorella P, Loiodice F, Laghezza A, Babini E, Zifarelli G, Pusch M, Camerino DC.

Proc Natl Acad Sci U S A. 2008 Jan 29;105(4):1369-73. doi: 10.1073/pnas.0708977105. Epub 2008 Jan 23.


Mechanism of interaction of niflumic acid with heterologously expressed kidney CLC-K chloride channels.

Picollo A, Liantonio A, Babini E, Camerino DC, Pusch M.

J Membr Biol. 2007 Apr;216(2-3):73-82. Epub 2007 Jul 21.


Ion channel pharmacology.

Camerino DC, Tricarico D, Desaphy JF.

Neurotherapeutics. 2007 Apr;4(2):184-98. Review.


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.


A new benzoxazine compound blocks KATP channels in pancreatic beta cells: molecular basis for tissue selectivity in vitro and hypoglycaemic action in vivo.

Rolland JF, Tricarico D, Laghezza A, Loiodice F, Tortorella V, Camerino DC.

Br J Pharmacol. 2006 Dec;149(7):870-9. Epub 2006 Oct 23.


Activation and inhibition of kidney CLC-K chloride channels by fenamates.

Liantonio A, Picollo A, Babini E, Carbonara G, Fracchiolla G, Loiodice F, Tortorella V, Pusch M, Camerino DC.

Mol Pharmacol. 2006 Jan;69(1):165-73. Epub 2005 Oct 21.


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.


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.


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