CNR Institutional Research Information System

SABATELLI, PATRIZIA ANNA
 Distribuzione geografica
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Continente #
AS - Asia 1.948
NA - Nord America 1.658
EU - Europa 482
SA - Sud America 450
Continente sconosciuto - Info sul continente non disponibili 46
AF - Africa 40
OC - Oceania 2
Totale 4.626
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Nazione #
US - Stati Uniti d'America 1.578
SG - Singapore 907
BR - Brasile 338
CN - Cina 333
VN - Vietnam 230
HK - Hong Kong 227
IT - Italia 149
FR - Francia 129
BD - Bangladesh 57
DE - Germania 45
KR - Corea 44
GB - Regno Unito 39
IN - India 37
AR - Argentina 35
CA - Canada 32
NL - Olanda 31
CO - Colombia 25
ID - Indonesia 24
FI - Finlandia 23
EC - Ecuador 22
JP - Giappone 21
MX - Messico 17
ZA - Sudafrica 15
IQ - Iraq 14
CL - Cile 12
RU - Federazione Russa 9
PL - Polonia 8
SA - Arabia Saudita 8
VE - Venezuela 8
IL - Israele 6
SE - Svezia 6
AT - Austria 5
CR - Costa Rica 5
ES - Italia 5
TR - Turchia 5
UA - Ucraina 5
DZ - Algeria 4
EE - Estonia 4
HN - Honduras 4
JM - Giamaica 4
JO - Giordania 4
KE - Kenya 4
MY - Malesia 4
NP - Nepal 4
RO - Romania 4
AE - Emirati Arabi Uniti 3
CZ - Repubblica Ceca 3
EG - Egitto 3
MA - Marocco 3
PA - Panama 3
PK - Pakistan 3
PY - Paraguay 3
TN - Tunisia 3
TW - Taiwan 3
AL - Albania 2
AU - Australia 2
BO - Bolivia 2
BY - Bielorussia 2
DO - Repubblica Dominicana 2
GE - Georgia 2
GT - Guatemala 2
GY - Guiana 2
KZ - Kazakistan 2
NI - Nicaragua 2
PE - Perù 2
PH - Filippine 2
PT - Portogallo 2
RS - Serbia 2
SC - Seychelles 2
SK - Slovacchia (Repubblica Slovacca) 2
SV - El Salvador 2
TH - Thailandia 2
TT - Trinidad e Tobago 2
UZ - Uzbekistan 2
AG - Antigua e Barbuda 1
AO - Angola 1
BE - Belgio 1
BH - Bahrain 1
BJ - Benin 1
CI - Costa d'Avorio 1
DK - Danimarca 1
ET - Etiopia 1
GA - Gabon 1
GR - Grecia 1
HR - Croazia 1
HT - Haiti 1
IE - Irlanda 1
KG - Kirghizistan 1
LV - Lettonia 1
MQ - Martinica 1
MT - Malta 1
NG - Nigeria 1
PR - Porto Rico 1
PS - Palestinian Territory 1
QA - Qatar 1
TC - Turks e Caicos 1
UY - Uruguay 1
XK - ???statistics.table.value.countryCode.XK??? 1
Totale 4.581
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Città #
Santa Clara 645
Singapore 510
Hong Kong 224
San Jose 141
Ashburn 131
Hefei 111
Lauterbourg 97
Ho Chi Minh City 77
Beijing 59
Hanoi 59
Dallas 46
Seoul 43
Los Angeles 33
São Paulo 33
New York 19
Pavia 19
Helsinki 18
Frankfurt am Main 16
Da Nang 15
Buffalo 13
Milan 13
Rio de Janeiro 13
Rome 13
Tokyo 13
Chicago 12
Haiphong 12
Brooklyn 10
Phoenix 10
Chennai 8
Düsseldorf 8
Guayaquil 8
Montreal 8
Orem 8
The Dalles 8
Belo Horizonte 7
Boston 7
Denver 7
Johannesburg 7
London 7
Minamishinagawa 7
Quito 7
Atlanta 6
Baghdad 6
Biên Hòa 6
Campinas 6
Council Bluffs 6
Houston 6
Philadelphia 6
Queens 6
Riyadh 6
São Bernardo do Campo 6
Toronto 6
Bexley 5
Brasília 5
Cleveland 5
Indianapolis 5
Nuremberg 5
Sorocaba 5
The Bronx 5
Thái Bình 5
Vũng Tàu 5
Amman 4
Amsterdam 4
Bengaluru 4
Bogotá 4
Bologna 4
Bắc Giang 4
Cincinnati 4
Falkenstein 4
Fortaleza 4
Guangzhou 4
Guarulhos 4
La Paz 4
Lappeenranta 4
Lexington 4
Medellín 4
Miami 4
North Charleston 4
Pelotas 4
Poplar 4
Recife 4
Stockholm 4
Ahmedabad 3
Bragança Paulista 3
Curitiba 3
Detroit 3
Florence 3
Ha Long 3
Hyderabad 3
Jakarta 3
Lấp Vò 3
Manaus 3
Manchester 3
Nairobi 3
Naples 3
New Haven 3
Nova Iguaçu 3
Orlando 3
Porto Alegre 3
Quận Bình Thạnh 3
Totale 2.751
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Nome #
Collagen VI Deficiency Impairs Tendon Fibroblasts Mechanoresponse in Ullrich Congenital Muscular Dystrophy 125
Emerin increase in regenerating muscle fibers, 86
Alisporivir rescues defective mitochondrial respiration in Duchenne muscular dystrophy 82
Interleukin-6 neutralization ameliorates symptoms in prematurely aged mice 82
Dysferlin in a hyperCKaemic patient with caveolin 3 mutation and in C2C12 cells after p38 MAP kinase inhibition. 80
Lamin A-dependent heterochromatin dynamics in control and progeria syndrome fibroblasts, 74
Elevated TGF beta 2 serum levels in Emery-Dreifuss Muscular Dystrophy: Implications for myocyte and tenocyte differentiation and fibrogenic processes 70
Collagen VI ablation in zebrafish causes neuromuscular defects during developmental and adult stages 67
Desmin and Plectin Recruitment to the Nucleus and Nuclei Orientation Are Lost in Emery-Dreifuss Muscular Dystrophy Myoblasts Subjected to Mechanical Stimulation 65
Collagen VI is required for the structural and functional integrity of the neuromuscular junction 63
Urine-Derived Stem Cells Express 571 Neuromuscular Disorders Causing Genes, Making Them a Potential in vitro Model for Rare Genetic Diseases 63
Tendon Extracellular Matrix Remodeling and Defective Cell Polarization in the Presence of Collagen VI Mutations. 61
Ambra1 deficiency impairs mitophagy in skeletal muscle 59
Early Morphological Changes of the Rectus Femoris Muscle and Deep Fascia in Ullrich Congenital Muscular Dystrophy 59
Effect on collagen VI extra-cellular assembly of COL6A1 and COL6A2 C-terminal mutationsi in Ullrich congenital muscular dystrophy. 56
Collagen VI myopathies: from the animal model to the clinical trial 56
Serum- and glucocorticoid- inducible kinase 2, SGK2, is a novel autophagy regulator and modulates platinum drugs response in cancer cells 54
3D ECM-rich environment sustains the identity of naive human iPSCs 53
Protein aggregates and autophagy involvement in a family with a mutation in Z-band alternatively spliced PDZ-motif protein. 52
Collagen VI-NG2 axis in human tendon fibroblasts under conditions mimicking injury response 51
Autosomal recessive Bethlem myopathy. 51
Multimerin-2 maintains vascular stability and permeability 51
A novel murine model for arrhythmogenic cardiomyopathy points to a pathogenic role of Wnt signalling and miRNA dysregulation 50
NIM811, a cyclophilin inhibitor without immunosuppressive activity, is beneficial in collagen VI congenital muscular dystrophy models 49
Lamin A N-terminal phosphorylation is associated with myoblast activation: impairment in Emery-Dreifuss muscular dystrophy, 49
New Clinical and Immunofluoresence Data of Collagen VI-Related Myopathy: A Single Center Cohort of 69 Patients 48
Treatment with a triazole inhibitor of the mitochondrial permeability transition pore fully corrects the pathology of sapje zebrafish lacking dystrophin 48
Altered expression of the MCSP/NG2 chondroitin sulfate proteoglycan in collagen VI deficiency, 45
Altered lamin A/C phosphorylation in Emery-Dreifuss muscle, 45
Homozygous Recessive Versican Missense Variation Is Associated With Early Teeth Loss in a Pakistani Family 44
Collagen VI in the Musculoskeletal System 44
114th ENMC International Workshop on Congenital Muscular Dystrophy (CMD) 17-19 January 2003, Naarden, The Netherlands: (8th Workshop of the International Consortium on CMD; 3rd Workshop of the MYO-CLUSTER project GENRE). 44
Lamin A N-terminal phosphorylation is associated with myoblast activation: impairment in Emery-Dreifuss muscular dystrophy. 44
Melanocytes from Patients Affected by Ullrich Congenital Muscular Dystrophy and Bethlem Myopathy have Dysfunctional Mitochondria That Can be Rescued with Cyclophilin Inhibitors. 43
Sustained oral spermidine supplementation rescues functional and structural defects in COL6-deficient myopathic mice 42
Genetic ablation of cyclophilin D rescues mitochondrial defects and prevents muscle apoptosis in collagen VI myopathic mice 42
Alopecia in Patients with Collagen VI-Related Myopathies: A Novel/Unrecognized Scalp Phenotype 41
Mitochondrial dysfunction in the pathogenesis of Ullrich congenital muscular dystrophy and prospective therapy with cyclosporins. 41
EMILIN-1 deficiency induces elastogenesis and vascular cell defects, 40
Laminopathies: involvement of structural nuclear proteins in the pathogenesis of an increasing number of human diseases, 40
Ultrastructural defects of collagen VI filaments in an Ullrich syndrome patient with loss of the alpha3(VI) N10-N7 domains, 39
Effect of mechanical strain on the collagen VI pericellular matrix in anterior cruciate ligament fibroblasts 39
Clinical and functional characterization of a long survivor congenital titinopathy patient with a novel metatranscript-only titin variant 39
Laminopathies: a chromatin affair, 38
Tendon Extracellular Matrix Alterations in Ullrich Congenital Muscular Dystrophy 37
EMILIN1/alpha9beta1 Integrin Interaction Is Crucial in Lymphatic Valve Formation and Maintenance. 37
Dominant and recessive COL6A1 mutations in Ullrich scleroatonic muscular dystrophy, 37
Autophagy activation in COL6 myopathic patients by a low-protein-diet pilot trial 36
Hyper-CK-emia as the sole manifestation of myotonic dystrophy type 2, 36
The myotonic dystrophy type 2 (DM2) gene product zinc finger protein 9 (ZNF9) is associated with sarcomeres and normally localized in DM2 patients' muscles. 36
Identification and characterization of novel collagen VI non-canonical splicing mutations causing ullrich congenital muscular dystrophy. 36
Extracellular matrix and nuclear abnormalities in skeletal muscle of a patient with Walker-Warburg syndrome caused by POMT1 mutation 36
Nuclear envelope proteins and chromatin arrangem ent: a pathogenic mechanism for laminopathies, 36
Tumor Necrosis Factor Receptor SF10A (TNFRSF10A) SNPs Correlate With Corticosteroid Response in Duchenne Muscular Dystrophy 36
Critical evaluation of the use of cell cultures for inclusion in clinical trials of patients affected by Collagen VI myopathies. 35
Mutations in COL6A3 cause severe and mild phenotypes of Ullrich congenital muscular dystrophy 35
Cyclosporine A in Ullrich congenital muscular dystrophy: long-term results 35
Detection of mitochondrial defects in collagen VI deficient muscle cultures from UCMD patients and Col6a1 knockout mice: an ultrastructural study, 34
Identification of a deep intronic mutation in the COL6A2 gene by a novel custom oligonucleotide CGH array designed to explore allelic and genetic heterogeneity in collagen VI-related myopathies. 34
Alterations of nuclear envelope and chromatin organization in mandibuloacral dysplasia, a rare form of laminopathy, 34
Monoamine oxidase inhibition prevents mitochondrial dysfunction and apoptosis in myoblasts from patients with collagen VI myopathies 34
Effects on Collagen VI mrna Stability and Microfibrillar Assembly of Three COL6A2 Mutations in Two Families with Ullrich Congenital Muscular Dystrophy 34
Expression of the Collagen VI alpha5 and alpha6 Chains in Normal Human Skin and in Skin of Patients with Collagen VI-Related Myopathies 33
Dystrophin levels as low as 30% are sufficient to avoid muscular dystrophy in the human. 33
Differential and restricted expression of novel collagen VI chains in mouse. 33
POMT2 mutations cause alpha-dystroglycan hypoglycosylation and Walker-Warburg syndrome, 33
Defective collagen VI alpha 6 chain expression in the skeletal muscle of patients with collagen VI-related myopathies 33
Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiency. 33
Ullrich scleroatonic muscular dystrophy is caused by recessive mutations in collagen type VI 33
Immunocytochemistry of nuclear domains and Emery-Dreifuss muscular dystrophy pathophysiology 32
Expression of collagen VI alpha5 and alpha6 chains in human muscle and in Duchenne muscular dystrophy-related muscle fibrosis. 32
Effect on collagen VI extra-cellular assembly of COL6A1 and COL6A2 C-terminal mutationsi in Ullrich congenital muscular dystrophy 32
PCNA staining pattern is altered in Emery-Dreifuss fibroblasts. 32
Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration 32
The epg5 knockout zebrafish line: a model to study Vici syndrome 32
Ultrastructural changes in muscle cells of patients with collagen VI-related myopathies. 32
Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies. 32
Characterization of a rare case of Ullrich congenital muscular dystrophy due to truncating mutations within the COL6A1 gene C-Terminal domain: a case report 32
Properties of Ca(2+) transport in mitochondria of Drosophila melanogaster 32
Preclinical PK and PD Studies on 2'-O-Methyl-phosphorothioate RNA Antisense Oligonucleotides in the mdx Mouse Model. 32
Cationic PMMA nanoparticles bind and deliver antisense oligoribonucleotides allowing restoration of dystrophin expression in the mdx mouse. 31
Macrophages: A minimally invasive tool for monitoring collagen VI myopathies. 31
In vivo biodistribution of non-viral systems for oligoribonucleotides delivery. 30
Emilin1 deficiency causes structural and functional defects of lymphatic vasculature 30
Genetic characterization in symptomatic female DMD carriers: lack of relationship between X-inactivation, transcriptional DMD allele balancing and phenotype. 30
Collagen type VI deficiency disrupts basal lamina-extracellular matrix binding in Ullrich congenital muscular dysystrophy and COL6A1 null mutant skeletal muscle 30
Heterochromatin dynamics in hutchinson-gilford progeria sindrome fibroblasts, 30
Autosomal recessive myosclerosis myopathy is a collagen VI disorder. 30
Failure of lamin A/C to functionally assemble in R482L mutated familial partial lipodystrophy fibroblasts: altered intermolecular interaction with emerin and implications for gene transcription. 30
Molecular analysis of COL6 genes in patients with Betlem myopaty and Ullrich congenital muscular dystrofy. 29
Chromatin remodelling is involved in the pathogenesis of the mandibulocral dyspasia (MADA), 29
Aggresome-autophagy involvement in a sarcopenic patient with rigid spine syndrome and a p.C150R mutation in FHL1 gene 29
Congenital myopathy with hanging big toe due to homozygous myopalladin (MYPN) mutation 29
Involvement of cytoskeletal and extracellular matrix structural components in LGND21 muscle: report of one case, 29
At the nucleus of the problem: nuclear proteins and disease 29
Improving clinical trial design for Duchenne muscular dystrophy 29
Muscle interstitial fibroblasts are the main source of collagen VI synthesis in skeletal muscle: implications for congenital muscular dystrophy types Ullrich and Bethlem. 28
Novel collagen VI alpha chains distribution in murine skeletal muscle: possible implications for neuromuscular disorders 28
The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects in Col6a1(-/-) myopathic mice. 28
Melanocytes-A novel tool to study mitochondrial dysfunction in Duchenne muscular dystrophy 28
Totale 4.222
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Categoria #
all - tutte 16.834
article - articoli 14.822
book - libri 0
conference - conferenze 0
curatela - curatele 0
other - altro 0
patent - brevetti 0
selected - selezionate 0
volume - volumi 0
Totale 31.656
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2023/20249 0 0 0 0 0 0 0 0 6 0 1 2
2024/20251.785 0 6 110 71 530 166 23 74 41 63 368 333
2025/20262.254 76 243 239 367 409 89 335 142 120 71 90 73
2026/2027578 167 113 298 0 0 0 0 0 0 0 0 0
Totale 4.626