IRIS
DESAPHY, Jean Francois
 Distribuzione geografica
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Continente #
NA - Nord America 13.751
AS - Asia 4.744
EU - Europa 3.998
SA - Sud America 1.347
Continente sconosciuto - Info sul continente non disponibili 610
AF - Africa 213
OC - Oceania 11
Totale 24.674
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Nazione #
US - Stati Uniti d'America 13.458
SG - Singapore 1.961
IT - Italia 1.300
BR - Brasile 1.076
CN - Cina 1.043
HK - Hong Kong 536
SE - Svezia 484
RU - Federazione Russa 451
DE - Germania 414
FR - Francia 304
VN - Vietnam 295
UA - Ucraina 280
FI - Finlandia 263
GB - Regno Unito 247
BD - Bangladesh 240
IN - India 162
CA - Canada 149
ID - Indonesia 98
AR - Argentina 97
CI - Costa d'Avorio 59
MX - Messico 58
IQ - Iraq 55
TR - Turchia 53
ZA - Sudafrica 42
EC - Ecuador 40
NL - Olanda 40
VE - Venezuela 38
CO - Colombia 37
BE - Belgio 36
JP - Giappone 36
PL - Polonia 36
PK - Pakistan 32
ES - Italia 30
SA - Arabia Saudita 28
CR - Costa Rica 25
IE - Irlanda 24
PH - Filippine 21
AT - Austria 20
UZ - Uzbekistan 20
MY - Malesia 19
EG - Egitto 18
MA - Marocco 18
CL - Cile 17
NP - Nepal 16
PY - Paraguay 16
JO - Giordania 15
LT - Lituania 15
TN - Tunisia 15
IR - Iran 13
KE - Kenya 12
AE - Emirati Arabi Uniti 11
ET - Etiopia 11
KZ - Kazakistan 11
HN - Honduras 10
JM - Giamaica 10
DO - Repubblica Dominicana 9
KR - Corea 9
TH - Thailandia 9
AU - Australia 8
DZ - Algeria 8
OM - Oman 8
PE - Perù 8
TT - Trinidad e Tobago 8
UY - Uruguay 8
AL - Albania 7
IL - Israele 7
LB - Libano 7
CZ - Repubblica Ceca 6
AZ - Azerbaigian 5
BO - Bolivia 5
LY - Libia 5
NI - Nicaragua 5
PA - Panama 5
PS - Palestinian Territory 5
AM - Armenia 4
CG - Congo 4
CH - Svizzera 4
EE - Estonia 4
KG - Kirghizistan 4
RO - Romania 4
SN - Senegal 4
AO - Angola 3
BG - Bulgaria 3
DK - Danimarca 3
GY - Guiana 3
HU - Ungheria 3
NG - Nigeria 3
NZ - Nuova Zelanda 3
PT - Portogallo 3
RS - Serbia 3
SC - Seychelles 3
SV - El Salvador 3
SY - Repubblica araba siriana 3
TW - Taiwan 3
BB - Barbados 2
BN - Brunei Darussalam 2
GA - Gabon 2
GE - Georgia 2
GT - Guatemala 2
KW - Kuwait 2
Totale 24.026
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Città #
Ashburn 1.543
Fairfield 1.155
Singapore 1.013
San Jose 828
Chandler 778
Woodbridge 747
Houston 651
Jacksonville 613
Hong Kong 533
Dallas 522
Milan 464
Cambridge 435
Seattle 433
Wilmington 389
Nyköping 385
Ann Arbor 373
Beijing 307
Council Bluffs 297
New York 270
Los Angeles 250
Lauterbourg 182
Roxbury 167
Lawrence 166
Bari 163
Santa Clara 148
Nanjing 147
Rome 136
Boardman 122
Princeton 114
Des Moines 106
São Paulo 104
Ho Chi Minh City 97
Helsinki 88
Inglewood 88
San Diego 72
Brooklyn 61
Buffalo 60
Columbus 58
Abidjan 57
Chicago 55
Hanoi 54
Jakarta 53
Atlanta 52
London 52
San Francisco 51
Phoenix 49
Shenyang 43
Denver 42
Hebei 42
Jiaxing 41
Toronto 41
Montreal 40
Munich 38
Nanchang 37
Dong Ket 34
Figino 34
Falkenstein 33
Washington 33
Brussels 32
Moscow 32
Brasília 31
Orem 31
Tokyo 31
Chennai 30
Rio de Janeiro 30
Dearborn 29
Frankfurt am Main 28
Warsaw 28
Turku 27
Turin 24
Changsha 23
The Dalles 23
Tianjin 23
Dhaka 22
Naples 22
Nuremberg 22
Falls Church 21
Curitiba 20
Johannesburg 20
North Bergen 20
Paris 20
Pune 20
Baghdad 19
Boston 19
Dublin 19
San Mateo 19
Guangzhou 18
Salvador 18
Stockholm 18
Charlotte 17
Guayaquil 17
San José 17
Augusta 16
Mexico City 16
Ankara 15
Belo Horizonte 15
Grottaglie 15
Miami 15
Poplar 15
Shanghai 15
Totale 15.858
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Nome #
Autonomic nervous response following rat hindlimb unloading: Investigation on some peripheral districts 862
The analysis of myotonia congenita mutations discloses functional clusters of amino acids within CBS2 domain and C-terminal peptide of ClC-1 channel 320
Involvement of voltage-gated sodium channels blockade in the analgesic effects of orphenadrine 268
Effects of Nandrolone in the counteraction of skeletal muscle atrophy in a mouse model of muscle disuse: molecular biology and functional evaluation 255
ClC-1 chloride channels: state-of-the-art research and future challenges 233
Multidisciplinary study of a new ClC-1 mutation causing myotonia congenita: a paradigm to understand and treat ion channelopathies 220
Statin-induced myotoxicity is exacerbated by aging: A biophysical and molecular biology study in rats treated with atorvastatin 218
Mapping ligand binding pockets in ClC-1 channels through an integrated in silico and experimental approach using anthracene-9-carboxylic acid and niflumic acid 215
Pharmacogenetics of myotonic hNav1.4 sodium channel variants situated near the fast inactivation gate 215
Antioxidant treatment of hindlimb-unloaded mouse counteracts fiber type transition but not atrophy of disused muscles 214
In vivo longitudinal study of rodent skeletal muscle atrophy using ultrasonography 212
Clinical, Molecular, and Functional Characterization of CLCN1 Mutations in Three Families with Recessive Myotonia Congenita 211
Increased sodium channel use-dependent inhibition by a new potent analogue of tocainide greatly enhances in vivo antimyotonic activity 206
Elucidating the Contribution of Skeletal Muscle Ion Channels to Amyotrophic Lateral Sclerosis in search of new therapeutic options 206
ClC-1 mutations in myotonia congenita patients: insights into molecular gating mechanisms and genotype-phenotype correlation 200
Adaptation of mouse skeletal muscle to long-term microgravity in the MDS mission 194
Characterization of Student Drinking Behaviors at the Beginning of the First Academic Year at One University in Southern Italy 194
Effects of a new potent analog of tocainide on hNav1.7 sodium channels and in vivo neuropathic pain models 192
A large cohort of myotonia congenita probands: novel mutations and a high-frequency mutation region in exons 4 and 5 of the CLCN1 gene. 191
Bioisosteric Modification of To042: Synthesis and Evaluation of Promising Use-Dependent Inhibitors of Voltage-Gated Sodium Channels 187
Chaperone activity of niflumic acid on ClC-1 chloride channel mutants causing myotonia congenita 174
Change of chloride ion channel conductance is an early event of slow-to-fast fibre type transition during unloading-induced muscle disuse 174
Coexistence of CLCN1 and SCN4A mutations in one family suffering from myotonia 172
Risk of myopathy in patients in therapy with statins: Identification of biological markers in a pilot study 172
Blockade by cAMP of native sodium channels of adult rat skeletal muscle fibers 171
A novel KCNA1 mutation in a patient with paroxysmal ataxia, myokymia, painful contractures and metabolic dysfunctions 171
Translational approach to address therapy in myotonia permanens due to a new SCN4A mutation 169
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 169
Hydroxylated analogs of mexiletine as tools for structural-requirements investigation of the sodium channel blocking activity 168
Safinamide's potential in treating nondystrophic myotonias: Inhibition of skeletal muscle voltage-gated sodium channels and skeletal muscle hyperexcitability in vitro and in vivo 167
A new benzylated proline-like tocainide derivative is a potent use-dependent skeletal muscle Na+ channel blocker with a potential anti-myotonic activity 164
Effects of Benzothiazolamines on Voltage-Gated Sodium Channels 158
Analysis by two-dimensional Blue Native/SDS-PAGE of membrane protein alterations in rat soleus muscle after hindlimb unloading 154
Effect of rat hindlimb suspension on cable properties, ionic conductances and fiber type composition of slow-twitch muscle. 152
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 152
Inhibition of voltage-gated sodium channels by sumatriptan bioisosteres 150
In vivo evaluation of antimyotonic efficacy of β-adrenergic drugs in a rat model of myotonia 146
Inhibitory effect of internal sodium and Hepes on the voltage-dependent potassium conductance of rat Leydig cells 145
Increased sarcolemma chloride conductance as one of the mechanisms of action of carbonic anhydrase inhibitors in muscle excitability disorders 145
Fiber type-related changes in rat skeletal muscle calcium homeostasis during aging and restoration by growth hormone 144
Therapeutic approaches to genetic ion channelopathies and perspectives in drug discovery 142
Statins and fenofibrate affect skeletal muscle chloride conductance in rats by different impairing ClC-1 channel regulation and expression 141
Antioxidant treatment of hindlimb-unloaded mouse in vivo counteracts fiber type transition but not atrophy of disused skeletal muscles 141
Synthesis and Evaluation of Voltage-Gated Sodium Channel Blocking Pyrroline Derivatives Endowed with Both Antiarrhythmic and Antioxidant Activities 141
Emerging roles for ion channels in ovarian cancer: Pathomechanisms and pharmacological treatment 141
Redox homeostasis, oxidative stress and disuse muscle atrophy 139
Functional characterization of a C-terminal Nav1.4 mutation found in a patient presenting with myotonia and congenital myasthenia syndrome 136
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 134
New potent mexiletine and tocainide analogues evaluated in vivo and in vitro as antimyotonic agents on the myotonic ADR mouse 134
Is oxidative stress a cause or consequence of disuse muscle atrophy in mice? A proteomic approach in hindlimb unloaded mice 132
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 132
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 132
ECM Composition Differentially Regulates Intracellular and Extracellular pH in Normal and Cancer Pancreatic Duct Epithelial Cells 131
Age-dependent effects of disuse on electrophysiogical parameters and calcium homeostasis in the soleus muscle of hindlimb-unloaded mice 131
Swelling and cAMP on hyperpolarization-activated Cl- conductance in rat Leydig cells 129
Ion channels in muscle and cardiac hereditary diseases: from gene dysfunction to pharmacological therapy 129
Use-Dependent block of voltage-gated sodium channels by orphenadrine through binding at the local anesthetic receptor 129
Missense and Non-Missense Lamin A/C Gene Mutations Are Similarly Associated with Major Arrhythmic Cardiac Events: A 20-Year Single-Centre Experience 128
Functional characterization of ClC-1 mutations from patients affected by recessive myotonia congenita presenting with different clinical phenotypes. 128
Effect of Mexiletine on transitory depression of compound motor action potential in recessive myotonia congenita 127
Molecular determinants of state dependent block of voltage-gated sodium channels by pilsicainide 126
Muscle loading modulates aquaporin-4 expression in skeletal muscle 125
Effects of GH secretagogues on resting chloride conductance of aged rat skeletal muscle 125
Taurine and skeletal muscle disorders 124
Taurine and skeletal muscle ion channels 122
Stereospecific Synthesis of ‘‘para-Hydroxymexiletine’’ and Sodium Channel Blocking Activity Evaluation 122
Ion channels gene expression analysis in myotonia congenita patients carrying ClC-1 chloride channel mutations 122
Effects of Pleiotrophin Overexpression on Mouse Skeletal Muscles in Normal Loading and in Actual and Simulated Microgravity 122
Ion Channel Gene Mutations Causing Skeletal Muscle Disorders: Pathomechanisms and Opportunities for Therapy 122
Combined modifications of mexiletine pharmacophores for new lead blockers of Nav1.4 channels 121
Skeletal muscle ClC-1 chloride channels in health and diseases 120
Pathomechanisms of a CLCN1 Mutation Found in a Russian Family Suffering From Becker's Myotonia 119
Molecular Determinants of Human Voltage-Gated Sodium Channels Blockade by Lubeluzole 118
Effect of cyclic AMP on the calcium-dependent potassium conductances of rat Leydig cells 117
Aromatic substituents on mexiletine chiral atom increase antimyotonic effect in vitro and in vivo 116
An olive oil-derived antioxidant mixture ameliorates the age-related decline of skeletal muscle function 116
Biophysical characterization of hClC-1 chloride channel mutations associated with myotonia congenita 114
Immersive and Non-Immersive Virtual Reality for Pain and Anxiety Management in Pediatric Patients with Hematological or Solid Cancer: A Systematic Review 113
Drug repurposing in skeletal muscle ion channelopathies 113
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. 113
Editorial: Recent advances in voltage-gated sodium channels, their pharmacology and related diseases 113
Molecular insights into the local anesthetic receptor within voltage-gated sodium channels using hydroxylated analogs of mexiletine 113
Privileged scaffold-based design to identify a novel drug-like 5-HT7 receptor-preferring agonist to target Fragile X syndrome 112
Effect of microgravity on gene expression in mouse brain 111
Ion Channel Involvement in Tumor Drug Resistance 111
The time course of disuse atrophy in a lsow and fast muscle of hindlimb unloaded mice 110
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 110
Structure-activity relationships in the sodium channel blocking activity of mexiletine analogues 109
Dramatic improvement of myotonia permanens with flecainide: a two-case report of a possible bench-to-bedside pharmacogenetics strategy 109
Poliprotect®, a Medical Device Made of Substances, Potently Protects the Human Esophageal Mucosa Challenged by Multiple Agents: Evidence from In Vitro and Ex Vivo Electrophysiological Models 108
Preclinical in vitro and in vivo evaluation of sodium channel blockers as possible alternative to mexiletine in the treatment of myotonia 108
Molecular dissection of lubeluzole use-dependent block of voltage-gated sodium channels discloses new therapeutic potentials 108
A guide to writing systematic reviews of rare disease treatments to generate FAIR-compliant datasets: Building a Treatabolome 108
Molecular determinants in mexiletine and tocainide structure to improve therapy of myotonia 107
Disuse of rat muscle in vivo reduces protein kinase C activity controlling the sarcolemma chloride conductance 107
Using occurrence data to evaluate extinction reveals a strong resilience of butterflies in a National Park of Southern Europe (Alta Murgia National Park) 107
Hydroxylation on the aromatic ring of mexiletine affects the block of voltage-gated sodium channels 106
New molecular requirements of Toc derivatives bearing a novel class of Nav1.4 blockers 106
Targeted Therapies for Skeletal Muscle Ion Channelopathies: Systematic Review and Steps Towards Precision Medicine 106
Potential benefits of taurine in the prevention of skeletal muscle impairment induced by disuse in the hindlimb-unloaded rat 105
Totale 15.471
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Categoria #
all - tutte 99.541
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 99.541
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2021/20221.058 0 0 0 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
2026/20271.977 203 385 568 821 0 0 0 0 0 0 0 0
Totale 24.674