IRIS
DESAPHY, Jean Francois
 Distribuzione geografica
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Continente #
NA - Nord America 12.162
AS - Asia 4.678
EU - Europa 3.874
SA - Sud America 1.300
AF - Africa 209
OC - Oceania 11
Continente sconosciuto - Info sul continente non disponibili 2
Totale 22.236
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Nazione #
US - Stati Uniti d'America 11.935
SG - Singapore 1.954
IT - Italia 1.233
BR - Brasile 1.053
CN - Cina 1.029
HK - Hong Kong 535
SE - Svezia 484
RU - Federazione Russa 447
DE - Germania 410
FR - Francia 299
VN - Vietnam 295
UA - Ucraina 277
FI - Finlandia 263
GB - Regno Unito 225
BD - Bangladesh 217
IN - India 160
CA - Canada 110
AR - Argentina 94
ID - Indonesia 94
CI - Costa d'Avorio 59
MX - Messico 57
IQ - Iraq 55
TR - Turchia 52
ZA - Sudafrica 42
NL - Olanda 39
JP - Giappone 36
EC - Ecuador 35
PL - Polonia 35
BE - Belgio 34
CO - Colombia 31
ES - Italia 30
PK - Pakistan 30
VE - Venezuela 30
SA - Arabia Saudita 26
IE - Irlanda 20
UZ - Uzbekistan 20
AT - Austria 19
PH - Filippine 19
EG - Egitto 18
MA - Marocco 18
CL - Cile 16
CR - Costa Rica 16
MY - Malesia 16
NP - Nepal 16
PY - Paraguay 16
JO - Giordania 15
TN - Tunisia 15
IR - Iran 13
KE - Kenya 12
ET - Etiopia 11
KZ - Kazakistan 11
AE - Emirati Arabi Uniti 10
LT - Lituania 10
DO - Repubblica Dominicana 9
KR - Corea 9
AU - Australia 8
DZ - Algeria 8
JM - Giamaica 8
OM - Oman 8
PE - Perù 8
AL - Albania 7
IL - Israele 7
LB - Libano 7
UY - Uruguay 7
CZ - Repubblica Ceca 6
TH - Thailandia 6
AZ - Azerbaigian 5
BO - Bolivia 5
HN - Honduras 5
LY - Libia 5
PS - Palestinian Territory 5
TT - Trinidad e Tobago 5
AM - Armenia 4
CH - Svizzera 4
KG - Kirghizistan 4
PA - Panama 4
RO - Romania 4
SN - Senegal 4
AO - Angola 3
BG - Bulgaria 3
CG - Congo 3
GY - Guiana 3
HU - Ungheria 3
NG - Nigeria 3
NZ - Nuova Zelanda 3
PT - Portogallo 3
RS - Serbia 3
SV - El Salvador 3
SY - Repubblica araba siriana 3
TW - Taiwan 3
BB - Barbados 2
DK - Danimarca 2
GA - Gabon 2
GE - Georgia 2
KW - Kuwait 2
LK - Sri Lanka 2
LV - Lettonia 2
MT - Malta 2
MU - Mauritius 2
NI - Nicaragua 2
Totale 22.204
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Città #
Ashburn 1.233
Fairfield 1.155
Singapore 1.010
Chandler 778
Woodbridge 747
Houston 647
Jacksonville 611
San Jose 556
Hong Kong 532
Dallas 513
Milan 451
Cambridge 435
Seattle 431
Wilmington 388
Nyköping 385
Ann Arbor 373
Beijing 299
New York 235
Los Angeles 230
Lauterbourg 182
Roxbury 167
Lawrence 166
Bari 162
Nanjing 147
Rome 131
Boardman 120
Princeton 114
Santa Clara 109
Des Moines 104
Ho Chi Minh City 97
Helsinki 88
Inglewood 88
São Paulo 88
Council Bluffs 83
San Diego 72
Brooklyn 59
Abidjan 57
Buffalo 55
Hanoi 54
Jakarta 53
London 51
Atlanta 47
San Francisco 46
Chicago 45
Shenyang 43
Hebei 42
Columbus 41
Jiaxing 41
Denver 39
Munich 38
Montreal 37
Nanchang 37
Phoenix 37
Dong Ket 34
Falkenstein 33
Figino 33
Moscow 32
Brussels 31
Orem 31
Tokyo 31
Brasília 30
Chennai 30
Dearborn 29
Rio de Janeiro 28
Warsaw 28
Frankfurt am Main 27
Toronto 27
Turku 27
Turin 24
Changsha 23
Tianjin 23
Washington 23
Dhaka 22
Nuremberg 22
The Dalles 22
Falls Church 21
Curitiba 20
Johannesburg 20
Paris 20
Pune 20
Baghdad 19
San Mateo 19
Dublin 18
Guangzhou 18
Salvador 18
Stockholm 18
Naples 16
Ankara 15
Augusta 15
Belo Horizonte 15
Boston 15
Grottaglie 15
Guayaquil 15
Mexico City 15
Poplar 15
Shanghai 15
Tashkent 15
Charlotte 14
Jinan 14
Amman 13
Totale 14.777
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Nome #
Autonomic nervous response following rat hindlimb unloading: Investigation on some peripheral districts 854
The analysis of myotonia congenita mutations discloses functional clusters of amino acids within CBS2 domain and C-terminal peptide of ClC-1 channel 286
Involvement of voltage-gated sodium channels blockade in the analgesic effects of orphenadrine 249
Effects of Nandrolone in the counteraction of skeletal muscle atrophy in a mouse model of muscle disuse: molecular biology and functional evaluation 246
ClC-1 chloride channels: state-of-the-art research and future challenges 227
Multidisciplinary study of a new ClC-1 mutation causing myotonia congenita: a paradigm to understand and treat ion channelopathies 210
Mapping ligand binding pockets in ClC-1 channels through an integrated in silico and experimental approach using anthracene-9-carboxylic acid and niflumic acid 210
Antioxidant treatment of hindlimb-unloaded mouse counteracts fiber type transition but not atrophy of disused muscles 206
Statin-induced myotoxicity is exacerbated by aging: A biophysical and molecular biology study in rats treated with atorvastatin 206
Clinical, Molecular, and Functional Characterization of CLCN1 Mutations in Three Families with Recessive Myotonia Congenita 203
Pharmacogenetics of myotonic hNav1.4 sodium channel variants situated near the fast inactivation gate 203
Elucidating the Contribution of Skeletal Muscle Ion Channels to Amyotrophic Lateral Sclerosis in search of new therapeutic options 201
In vivo longitudinal study of rodent skeletal muscle atrophy using ultrasonography 200
Increased sodium channel use-dependent inhibition by a new potent analogue of tocainide greatly enhances in vivo antimyotonic activity 197
ClC-1 mutations in myotonia congenita patients: insights into molecular gating mechanisms and genotype-phenotype correlation 194
Characterization of Student Drinking Behaviors at the Beginning of the First Academic Year at One University in Southern Italy 190
Adaptation of mouse skeletal muscle to long-term microgravity in the MDS mission 187
A large cohort of myotonia congenita probands: novel mutations and a high-frequency mutation region in exons 4 and 5 of the CLCN1 gene. 184
Effects of a new potent analog of tocainide on hNav1.7 sodium channels and in vivo neuropathic pain models 183
Bioisosteric Modification of To042: Synthesis and Evaluation of Promising Use-Dependent Inhibitors of Voltage-Gated Sodium Channels 175
Change of chloride ion channel conductance is an early event of slow-to-fast fibre type transition during unloading-induced muscle disuse 169
Coexistence of CLCN1 and SCN4A mutations in one family suffering from myotonia 166
Blockade by cAMP of native sodium channels of adult rat skeletal muscle fibers 162
Risk of myopathy in patients in therapy with statins: Identification of biological markers in a pilot study 161
Safinamide's potential in treating nondystrophic myotonias: Inhibition of skeletal muscle voltage-gated sodium channels and skeletal muscle hyperexcitability in vitro and in vivo 161
A novel KCNA1 mutation in a patient with paroxysmal ataxia, myokymia, painful contractures and metabolic dysfunctions 160
Chaperone activity of niflumic acid on ClC-1 chloride channel mutants causing myotonia congenita 159
Hydroxylated analogs of mexiletine as tools for structural-requirements investigation of the sodium channel blocking activity 157
Translational approach to address therapy in myotonia permanens due to a new SCN4A mutation 156
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 155
Effects of Benzothiazolamines on Voltage-Gated Sodium Channels 152
A new benzylated proline-like tocainide derivative is a potent use-dependent skeletal muscle Na+ channel blocker with a potential anti-myotonic activity 151
Analysis by two-dimensional Blue Native/SDS-PAGE of membrane protein alterations in rat soleus muscle after hindlimb unloading 148
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 145
Inhibition of voltage-gated sodium channels by sumatriptan bioisosteres 141
Fiber type-related changes in rat skeletal muscle calcium homeostasis during aging and restoration by growth hormone 140
Inhibitory effect of internal sodium and Hepes on the voltage-dependent potassium conductance of rat Leydig cells 139
Antioxidant treatment of hindlimb-unloaded mouse in vivo counteracts fiber type transition but not atrophy of disused skeletal muscles 138
Redox homeostasis, oxidative stress and disuse muscle atrophy 136
Therapeutic approaches to genetic ion channelopathies and perspectives in drug discovery 135
Statins and fenofibrate affect skeletal muscle chloride conductance in rats by different impairing ClC-1 channel regulation and expression 131
Synthesis and Evaluation of Voltage-Gated Sodium Channel Blocking Pyrroline Derivatives Endowed with Both Antiarrhythmic and Antioxidant Activities 130
Is oxidative stress a cause or consequence of disuse muscle atrophy in mice? A proteomic approach in hindlimb unloaded mice 129
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 129
New potent mexiletine and tocainide analogues evaluated in vivo and in vitro as antimyotonic agents on the myotonic ADR mouse 128
Increased sarcolemma chloride conductance as one of the mechanisms of action of carbonic anhydrase inhibitors in muscle excitability disorders 128
In vivo evaluation of antimyotonic efficacy of β-adrenergic drugs in a rat model of myotonia 127
Functional characterization of a C-terminal Nav1.4 mutation found in a patient presenting with myotonia and congenital myasthenia syndrome 127
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 126
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 126
Ion channels in muscle and cardiac hereditary diseases: from gene dysfunction to pharmacological therapy 125
Use-Dependent block of voltage-gated sodium channels by orphenadrine through binding at the local anesthetic receptor 125
Functional characterization of ClC-1 mutations from patients affected by recessive myotonia congenita presenting with different clinical phenotypes. 123
Emerging roles for ion channels in ovarian cancer: Pathomechanisms and pharmacological treatment 123
ECM Composition Differentially Regulates Intracellular and Extracellular pH in Normal and Cancer Pancreatic Duct Epithelial Cells 122
Molecular determinants of state dependent block of voltage-gated sodium channels by pilsicainide 121
Taurine and skeletal muscle ion channels 119
Muscle loading modulates aquaporin-4 expression in skeletal muscle 119
Effects of GH secretagogues on resting chloride conductance of aged rat skeletal muscle 118
Age-dependent effects of disuse on electrophysiogical parameters and calcium homeostasis in the soleus muscle of hindlimb-unloaded mice 118
Effect of Mexiletine on transitory depression of compound motor action potential in recessive myotonia congenita 118
Swelling and cAMP on hyperpolarization-activated Cl- conductance in rat Leydig cells 117
Effects of Pleiotrophin Overexpression on Mouse Skeletal Muscles in Normal Loading and in Actual and Simulated Microgravity 117
Taurine and skeletal muscle disorders 116
Ion Channel Gene Mutations Causing Skeletal Muscle Disorders: Pathomechanisms and Opportunities for Therapy 116
Combined modifications of mexiletine pharmacophores for new lead blockers of Nav1.4 channels 115
Effect of cyclic AMP on the calcium-dependent potassium conductances of rat Leydig cells 113
Molecular Determinants of Human Voltage-Gated Sodium Channels Blockade by Lubeluzole 112
Stereospecific Synthesis of ‘‘para-Hydroxymexiletine’’ and Sodium Channel Blocking Activity Evaluation 112
Ion channels gene expression analysis in myotonia congenita patients carrying ClC-1 chloride channel mutations 112
Pathomechanisms of a CLCN1 Mutation Found in a Russian Family Suffering From Becker's Myotonia 112
Skeletal muscle ClC-1 chloride channels in health and diseases 111
Missense and Non-Missense Lamin A/C Gene Mutations Are Similarly Associated with Major Arrhythmic Cardiac Events: A 20-Year Single-Centre Experience 110
Editorial: Recent advances in voltage-gated sodium channels, their pharmacology and related diseases 109
An olive oil-derived antioxidant mixture ameliorates the age-related decline of skeletal muscle function 108
Ion Channel Involvement in Tumor Drug Resistance 107
Effect of rat hindlimb suspension on cable properties, ionic conductances and fiber type composition of slow-twitch muscle. 106
Aromatic substituents on mexiletine chiral atom increase antimyotonic effect in vitro and in vivo 105
Drug repurposing in skeletal muscle ion channelopathies 104
Biophysical characterization of hClC-1 chloride channel mutations associated with myotonia congenita 104
Dramatic improvement of myotonia permanens with flecainide: a two-case report of a possible bench-to-bedside pharmacogenetics strategy 104
Effect of microgravity on gene expression in mouse brain 103
Molecular insights into the local anesthetic receptor within voltage-gated sodium channels using hydroxylated analogs of mexiletine 103
Disuse of rat muscle in vivo reduces protein kinase C activity controlling the sarcolemma chloride conductance 102
Molecular dissection of lubeluzole use-dependent block of voltage-gated sodium channels discloses new therapeutic potentials 102
Immersive and Non-Immersive Virtual Reality for Pain and Anxiety Management in Pediatric Patients with Hematological or Solid Cancer: A Systematic Review 101
Privileged scaffold-based design to identify a novel drug-like 5-HT7 receptor-preferring agonist to target Fragile X syndrome 101
Potential benefits of taurine in the prevention of skeletal muscle impairment induced by disuse in the hindlimb-unloaded rat 100
Anti-Angiogenic Activity of Drugs in Multiple Myeloma 99
Targeted Therapies for Skeletal Muscle Ion Channelopathies: Systematic Review and Steps Towards Precision Medicine 98
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. 97
Structure-activity relationships in the sodium channel blocking activity of mexiletine analogues 97
Pilsicainide and its oxymethylene analog: Facile alternative synthesis and in vitro testing on human skeletal muscle sodium channels 97
An olive oil–derived antioxidant mixture ameliorates the age-related decline of skeletal muscle function 97
Different ability of clenbuterol and salbutamol to block sodium channels predicts their therapeutic use in muscle excitability disorders 96
Molecular determinants in mexiletine and tocainide structure to improve therapy of myotonia 95
Preclinical in vitro and in vivo evaluation of sodium channel blockers as possible alternative to mexiletine in the treatment of myotonia 95
Therapeutic approaches to ion channel diseases 95
Recent advances in voltage-gated sodium channels, their pharmacology and related diseases 95
Musculoskeletal Features without Ataxia Associated with a Novel de novo Mutation in KCNA1 Impairing the Voltage Sensitivity of Kv1.1 Channel 95
Totale 14.532
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Categoria #
all - tutte 92.548
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 92.548
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2021/20221.302 53 183 8 94 57 76 48 64 89 102 211 317
2022/20232.082 303 185 139 200 228 303 22 261 332 12 52 45
2023/2024663 43 140 42 66 65 165 23 15 9 18 23 54
2024/20253.405 110 47 263 106 183 270 367 329 170 156 465 939
2025/20268.480 985 360 709 733 770 403 843 225 783 669 1.049 951
2026/2027146 146 0 0 0 0 0 0 0 0 0 0 0
Totale 22.843