IRIS
DESAPHY, Jean Francois
 Distribuzione geografica
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Continente #
NA - Nord America 8.168
EU - Europa 1.947
AS - Asia 1.088
SA - Sud America 43
AF - Africa 6
OC - Oceania 5
Continente sconosciuto - Info sul continente non disponibili 1
Totale 11.258
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Nazione #
US - Stati Uniti d'America 8.148
CN - Cina 654
SE - Svezia 471
SG - Singapore 327
DE - Germania 283
IT - Italia 276
UA - Ucraina 258
RU - Federazione Russa 196
FI - Finlandia 179
GB - Regno Unito 134
FR - Francia 68
BR - Brasile 35
VN - Vietnam 35
BE - Belgio 31
IN - India 28
CA - Canada 19
IE - Irlanda 17
NL - Olanda 13
IR - Iran 11
JP - Giappone 7
CZ - Repubblica Ceca 6
HK - Hong Kong 5
AT - Austria 4
EG - Egitto 4
ID - Indonesia 4
TR - Turchia 4
AR - Argentina 3
CL - Cile 3
KR - Corea 3
NZ - Nuova Zelanda 3
AU - Australia 2
LT - Lituania 2
MA - Marocco 2
PH - Filippine 2
RO - Romania 2
UZ - Uzbekistan 2
BG - Bulgaria 1
CH - Svizzera 1
CR - Costa Rica 1
DK - Danimarca 1
EC - Ecuador 1
EE - Estonia 1
EU - Europa 1
HU - Ungheria 1
IL - Israele 1
LA - Repubblica Popolare Democratica del Laos 1
LB - Libano 1
LV - Lettonia 1
NP - Nepal 1
PL - Polonia 1
QA - Qatar 1
TW - Taiwan 1
UY - Uruguay 1
Totale 11.258
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Città #
Fairfield 1.155
Chandler 784
Woodbridge 747
Jacksonville 609
Houston 607
Ashburn 549
Cambridge 435
Seattle 413
Nyköping 389
Wilmington 386
Ann Arbor 373
Singapore 247
Lawrence 167
Roxbury 167
Nanjing 146
Bari 134
Princeton 115
Beijing 108
Des Moines 103
Boardman 97
New York 93
Inglewood 88
San Diego 72
Los Angeles 47
Santa Clara 45
Shenyang 43
Hebei 42
Jiaxing 41
Nanchang 37
Dong Ket 34
Helsinki 31
Dearborn 29
Brussels 28
London 28
Tianjin 23
Changsha 22
Falls Church 20
San Mateo 19
Paris 18
Dublin 17
Pune 17
Guangzhou 16
Brooklyn 14
Shanghai 13
Toronto 13
Washington 13
Jinan 12
Montignoso 12
Wuhan 11
Ardabil 10
Augusta 10
Kunming 10
Hefei 8
Milan 8
Monmouth Junction 8
Redwood City 8
Ningbo 7
Norwalk 7
Zhengzhou 7
Brno 6
Leawood 6
San Francisco 6
Indiana 5
Mumbai 5
Nuremberg 5
Shenzhen 5
Tappahannock 5
Taranto 5
Campobello Di Mazara 4
Hong Kong 4
Hounslow 4
Marseille 4
Mottola 4
New Bedfont 4
Prescot 4
São Paulo 4
Tokyo 4
Trani 4
Triggiano 4
Altamura 3
Auburn Hills 3
Belém 3
Bologna 3
Brasília 3
Chicago 3
Chiswick 3
Chongqing 3
Clichy 3
Clifton 3
Falkenstein 3
Grottaglie 3
Hangzhou 3
Islington 3
Jinhua 3
Ottawa 3
Rome 3
Acton 2
Auckland 2
Baotou 2
Barletta 2
Totale 8.863
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Nome #
Multidisciplinary study of a new ClC-1 mutation causing myotonia congenita: a paradigm to understand and treat ion channelopathies 162
ClC-1 chloride channels: state-of-the-art research and future challenges 154
Effects of Nandrolone in the counteraction of skeletal muscle atrophy in a mouse model of muscle disuse: molecular biology and functional evaluation 142
Pharmacogenetics of myotonic hNav1.4 sodium channel variants situated near the fast inactivation gate 141
Mapping ligand binding pockets in ClC-1 channels through an integrated in silico and experimental approach using anthracene-9-carboxylic acid and niflumic acid 137
Involvement of voltage-gated sodium channels blockade in the analgesic effects of orphenadrine 134
Clinical, Molecular, and Functional Characterization of CLCN1 Mutations in Three Families with Recessive Myotonia Congenita 132
Statin-induced myotoxicity is exacerbated by aging: A biophysical and molecular biology study in rats treated with atorvastatin 126
Adaptation of mouse skeletal muscle to long-term microgravity in the MDS mission 125
Antioxidant treatment of hindlimb-unloaded mouse counteracts fiber type transition but not atrophy of disused muscles 123
Elucidating the Contribution of Skeletal Muscle Ion Channels to Amyotrophic Lateral Sclerosis in search of new therapeutic options 121
In vivo longitudinal study of rodent skeletal muscle atrophy using ultrasonography 120
Characterization of Student Drinking Behaviors at the Beginning of the First Academic Year at One University in Southern Italy 116
The analysis of myotonia congenita mutations discloses functional clusters of amino acids within CBS2 domain and C-terminal peptide of ClC-1 channel 114
A large cohort of myotonia congenita probands: novel mutations and a high-frequency mutation region in exons 4 and 5 of the CLCN1 gene. 112
ClC-1 mutations in myotonia congenita patients: insights into molecular gating mechanisms and genotype-phenotype correlation 112
Coexistence of CLCN1 and SCN4A mutations in one family suffering from myotonia 110
Increased rigidity of the chiral centre of tocainide favours stereoselectivity and use-dependent block of skeletal muscle Na+ channels enhancing the antimyotonic activity in vivo 110
Risk of myopathy in patients in therapy with statins: Identification of biological markers in a pilot study 108
Effects of a new potent analog of tocainide on hNav1.7 sodium channels and in vivo neuropathic pain models 106
Increased sodium channel use-dependent inhibition by a new potent analogue of tocainide greatly enhances in vivo antimyotonic activity 104
Redox homeostasis, oxidative stress and disuse muscle atrophy 102
Blockade by cAMP of native sodium channels of adult rat skeletal muscle fibers 100
Effects of Benzothiazolamines on Voltage-Gated Sodium Channels 100
Is oxidative stress a cause or consequence of disuse muscle atrophy in mice? A proteomic approach in hindlimb unloaded mice 99
A novel KCNA1 mutation in a patient with paroxysmal ataxia, myokymia, painful contractures and metabolic dysfunctions 99
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 98
Ion channels in muscle and cardiac hereditary diseases: from gene dysfunction to pharmacological therapy 96
Inhibition of voltage-gated sodium channels by sumatriptan bioisosteres 96
Change of chloride ion channel conductance is an early event of slow-to-fast fibre type transition during unloading-induced muscle disuse 95
Hydroxylated analogs of mexiletine as tools for structural-requirements investigation of the sodium channel blocking activity 95
In vivo evaluation of antimyotonic efficacy of β-adrenergic drugs in a rat model of myotonia 93
Fiber type-related changes in rat skeletal muscle calcium homeostasis during aging and restoration by growth hormone 93
Inhibitory effect of internal sodium and Hepes on the voltage-dependent potassium conductance of rat Leydig cells 92
Translational approach to address therapy in myotonia permanens due to a new SCN4A mutation 92
Taurine and skeletal muscle ion channels 91
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Swelling and cAMP on hyperpolarization-activated Cl- conductance in rat Leydig cells 89
Effect of cyclic AMP on the calcium-dependent potassium conductances of rat Leydig cells 89
Effect of Mexiletine on transitory depression of compound motor action potential in recessive myotonia congenita 88
null 87
Analysis by two-dimensional Blue Native/SDS-PAGE of membrane protein alterations in rat soleus muscle after hindlimb unloading 86
Molecular determinants of state dependent block of voltage-gated sodium channels by pilsicainide 84
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 84
Muscle loading modulates aquaporin-4 expression in skeletal muscle 83
Use-Dependent block of voltage-gated sodium channels by orphenadrine through binding at the local anesthetic receptor 80
Molecular Determinants of Human Voltage-Gated Sodium Channels Blockade by Lubeluzole 78
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Therapeutic approaches to genetic ion channelopathies and perspectives in drug discovery 77
Stereospecific Synthesis of ‘‘para-Hydroxymexiletine’’ and Sodium Channel Blocking Activity Evaluation 77
Dramatic improvement of myotonia permanens with flecainide: a two-case report of a possible bench-to-bedside pharmacogenetics strategy 76
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Taurine and skeletal muscle disorders 75
New potent mexiletine and tocainide analogues evaluated in vivo and in vitro as antimyotonic agents on the myotonic ADR mouse 75
Recent advances in voltage-gated sodium channels, their pharmacology and related diseases 75
Editorial: Recent advances in voltage-gated sodium channels, their pharmacology and related diseases 73
Molecular dissection of lubeluzole use-dependent block of voltage-gated sodium channels discloses new therapeutic potentials 73
null 72
Effect of microgravity on gene expression in mouse brain 72
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 72
18. Desaphy J.-F., De Luca A., George A.L. Jr. and D. Conte Camerino. Improved block of human skeletal muscle Na+ channel mutants by a new mexiletine derivative 70
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Ion channels gene expression analysis in myotonia congenita patients carrying ClC-1 chloride channel mutations 70
Molecular insights into the local anesthetic receptor within voltage-gated sodium channels using hydroxylated analogs of mexiletine 69
Statins and fenofibrate affect skeletal muscle chloride conductance in rats by different impairing ClC-1 channel regulation and expression 68
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Safinamide's potential in treating nondystrophic myotonias: Inhibition of skeletal muscle voltage-gated sodium channels and skeletal muscle hyperexcitability in vitro and in vivo 68
Potential targets for skeletal muscle impairment by hypogravity: basic characterization of resting ionic conductances and mechanical threshold of rat fast- and slow-twitch muscle fibers 66
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Functional characterization of a C-terminal Nav1.4 mutation found in a patient presenting with myotonia and congenital myasthenia syndrome 65
Therapeutic approaches to ion channel diseases 65
Functional characterization of ClC-1 mutations from patients affected by recessive myotonia congenita presenting with different clinical phenotypes. 64
Different ability of clenbuterol and salbutamol to block sodium channels predicts their therapeutic use in muscle excitability disorders 64
Comparison of excitability parameters and sodium channel behavior of fast- and slow-twitch rat skeletal muscles for the study of the effects of hindlimb suspension, a model of hypogravity. 63
Effect of rat hindlimb suspension on cable properties, ionic conductances and fiber type composition of slow-twitch muscle. 63
Skeletal muscle ClC-1 chloride channels in health and diseases 62
Synthesis and Evaluation of Voltage-Gated Sodium Channel Blocking Pyrroline Derivatives Endowed with Both Antiarrhythmic and Antioxidant Activities 62
Preclinical in vitro and in vivo evaluation of sodium channel blockers as possible alternative to mexiletine in the treatment of myotonia 61
Hydroxylation on the aromatic ring of mexiletine affects the block of voltage-gated sodium channels 60
Disuse of rat muscle in vivo reduces protein kinase C activity controlling the sarcolemma chloride conductance 60
Ion channel pharmacology 60
null 58
Gating of myotonic Na channel mutants defines the response to mexiletine and a potent derivative 58
Decrease in resting calcium and calcium entry associated with slow-to-fast transition in unloaded rat soleus muscle 58
Autonomic nervous response following rat hindlimb unloading: Investigation on some peripheral districts 57
Molecular determinants of human voltage-gated sodium channel blockade by lubeluzole 57
Aromatic substituents on mexiletine chiral atom increase antimyotonic effect in vitro and in vivo 57
Antioxidant treatment of hindlimb-unloaded mouse in vivo counteracts fiber type transition but not atrophy of disused skeletal muscles 57
Potential benefits of taurine in the prevention of skeletal muscle impairment induced by disuse in the hindlimb-unloaded rat 56
Structure-activity relationships in the sodium channel blocking activity of mexiletine analogues 56
Molecular determinants in mexiletine and tocainide structure to improve therapy of myotonia 55
Effects of GH secretagogues on resting chloride conductance of aged rat skeletal muscle 55
Effect of mexiletine on sea anemone toxin-induced noninactivating sodium channels of rat skeletal muscle, a model of sodium channel myotonia 55
Pilsicainide and its oxymethylene analog: Facile alternative synthesis and in vitro testing on human skeletal muscle sodium channels 54
A new benzylated proline-like tocainide derivative is a potent use-dependent skeletal muscle Na+ channel blocker with a potential anti-myotonic activity 54
Pathomechanisms of a CLCN1 Mutation Found in a Russian Family Suffering From Becker's Myotonia 53
Bioisosteric Modification of To042: Synthesis and Evaluation of Promising Use-Dependent Inhibitors of Voltage-Gated Sodium Channels 53
Expression of sarcolemmal ion channels in slow and fast-twitch muscles of rodents in simulated and actual microgravity 52
Totale 8.429
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Categoria #
all - tutte 54.836
article - articoli 0
book - libri 0
conference - conferenze 0
curatela - curatele 0
other - altro 0
patent - brevetti 0
selected - selezionate 0
volume - volumi 0
Totale 54.836
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2019/20201.178 0 0 0 0 0 0 276 191 283 132 234 62
2020/20212.030 217 88 167 204 207 101 171 106 173 289 178 129
2021/20221.302 53 183 8 94 57 76 48 64 89 102 211 317
2022/20232.098 305 188 140 202 230 305 22 263 334 12 52 45
2023/2024668 43 141 42 67 65 167 23 15 9 19 23 54
2024/2025984 111 47 264 106 184 271 1 0 0 0 0 0
Totale 11.817