BERTOLDI, Mariarita
 Distribuzione geografica
Continente #
EU - Europa 4.139
NA - Nord America 3.429
AS - Asia 2.954
SA - Sud America 412
AF - Africa 58
OC - Oceania 7
Continente sconosciuto - Info sul continente non disponibili 2
Totale 11.001
Nazione #
US - Stati Uniti d'America 3.369
RU - Federazione Russa 1.758
SG - Singapore 1.148
GB - Regno Unito 1.031
CN - Cina 1.026
BR - Brasile 337
HK - Hong Kong 286
IT - Italia 249
FR - Francia 208
VN - Vietnam 204
FI - Finlandia 198
DE - Germania 196
SE - Svezia 168
IE - Irlanda 149
KR - Corea 84
UA - Ucraina 56
CA - Canada 41
ID - Indonesia 36
AR - Argentina 31
JP - Giappone 31
IN - India 30
BD - Bangladesh 23
NL - Olanda 23
TR - Turchia 20
AT - Austria 19
BE - Belgio 17
ES - Italia 17
TG - Togo 15
BJ - Benin 14
PL - Polonia 14
MX - Messico 13
EC - Ecuador 11
CL - Cile 10
LT - Lituania 10
IQ - Iraq 9
JO - Giordania 9
UZ - Uzbekistan 9
MA - Marocco 8
ZA - Sudafrica 8
IL - Israele 7
PK - Pakistan 6
AE - Emirati Arabi Uniti 5
BG - Bulgaria 5
CO - Colombia 5
PY - Paraguay 5
SA - Arabia Saudita 5
AU - Australia 4
BO - Bolivia 4
RO - Romania 4
SK - Slovacchia (Repubblica Slovacca) 4
UY - Uruguay 4
CH - Svizzera 3
KE - Kenya 3
BH - Bahrain 2
DK - Danimarca 2
EG - Egitto 2
GE - Georgia 2
LB - Libano 2
NG - Nigeria 2
NZ - Nuova Zelanda 2
PA - Panama 2
PE - Perù 2
A2 - ???statistics.table.value.countryCode.A2??? 1
AF - Afghanistan, Repubblica islamica di 1
AL - Albania 1
AM - Armenia 1
AZ - Azerbaigian 1
BA - Bosnia-Erzegovina 1
BY - Bielorussia 1
CR - Costa Rica 1
DO - Repubblica Dominicana 1
DZ - Algeria 1
EU - Europa 1
GF - Guiana Francese 1
GR - Grecia 1
GY - Guiana 1
HR - Croazia 1
HU - Ungheria 1
JM - Giamaica 1
KG - Kirghizistan 1
KH - Cambogia 1
MU - Mauritius 1
NO - Norvegia 1
OM - Oman 1
PG - Papua Nuova Guinea 1
PS - Palestinian Territory 1
QA - Qatar 1
RW - Ruanda 1
SI - Slovenia 1
SN - Senegal 1
SV - El Salvador 1
SY - Repubblica araba siriana 1
TH - Thailandia 1
TN - Tunisia 1
VE - Venezuela 1
ZW - Zimbabwe 1
Totale 11.001
Città #
Southend 924
Moscow 610
Singapore 555
Jacksonville 405
Dallas 401
Chandler 313
Ashburn 312
Hong Kong 284
Woodbridge 226
Ann Arbor 198
Houston 192
Beijing 180
Dublin 149
Verona 122
Jinan 102
The Dalles 84
Shenyang 74
Wilmington 73
Lawrence 69
Los Angeles 69
Princeton 69
Nanjing 62
Helsinki 61
New York 58
Ho Chi Minh City 51
Buffalo 47
Hebei 44
Munich 44
Columbus 39
Changsha 36
Tianjin 35
São Paulo 34
Hanoi 32
Zhengzhou 32
Ningbo 31
Tokyo 29
Jakarta 27
London 27
Redondo Beach 26
Milan 22
Falls Church 21
Haikou 21
Chicago 20
Guangzhou 20
Hangzhou 20
Frankfurt am Main 19
Sindelfingen 19
Lappeenranta 18
Santa Clara 18
Taiyuan 18
Nanchang 17
Seattle 17
Brussels 16
Montreal 16
Nuremberg 16
Redmond 16
Taizhou 16
Lomé 15
Turku 15
Cotonou 14
Council Bluffs 14
Dong Ket 14
Lancaster 14
Jiaxing 13
Lanzhou 13
Norwalk 13
Denver 12
Fuzhou 12
Orem 12
Rome 12
Warsaw 12
Amsterdam 11
Rio de Janeiro 11
San Francisco 11
Seoul 11
Toronto 11
Boston 10
Brooklyn 10
Chennai 10
Phoenix 10
Amman 9
Boardman 9
Hải Dương 9
San Jose 9
Vienna 9
Haiphong 8
Kent 8
Shanghai 8
Tashkent 8
Brasília 7
Fairfield 7
Renton 7
Stockholm 7
Belo Horizonte 6
Campinas 6
Da Nang 6
Dhaka 6
Elora 6
Falkenstein 6
Ninh Bình 6
Totale 6.883
Nome #
Dimerization and Folding Processes of Treponema denticola Cystalysin: The Role of Pyridoxal 5'-Phosphate 242
Holo-and apocystalysin from Treponema denticola: two different conformations 238
Pyridoxal 5’-Phosphate Enzymes as Targets for Therapeutic Agents 212
A new molecular link between defective autophagy and erythroid abnormalities in chorea-acanthocytosis 198
The novel R347g pathogenic mutation Of aromatic amino acid decarboxylase provides additional molecular insights into enzyme catalysis and deficiency 195
Human wild-type alanine:glyoxylate aminotransferase and its naturally occurring G82E variant: functional properties and physiological implications 183
Resveratrol Induces Erythroid Maturation by Activating FOXO3 and Improves in Vivo Erythropoiesis in Normal and Beta -Thalassemic Mice 183
A quinonoid is an intermediate of oxidative deamination reaction catalyzed by Dopa decarboxylase 176
Artificial Ribonuclease A oligomers degrade double-stranded RNA with efficiency that increases as a function of the size of the oligomer 171
Pathogenic variants of human Aromatic L-Amino Acid Decarboxylase: evidences of misfolding in functionally active variants 171
An engineered folded PLP-bound monomer of Treponema denticola cystalysin reveals the effect of the dimeric structure on the catalytic properties of the enzyme 170
Dopa decarboxylase exhibits low pH half-transaminase and high pH oxidative deaminase activities toward serotonin (5-hydroxytryptamine) 169
Unique substrate specificity of ornithine aminotransferase from Toxoplasma gondii 163
Membrane association of peroxiredoxin-2 in red cells is mediated by the N-terminal cytoplasmic domain of band3 162
Structural properties of trimers and tetramers of ribonuclease A 160
Erythrocyte membrane changes of chorea-acanthocytosis are the result of altered Lyn kinase activity 160
Oxidative stress and b-thalasssemic erythroid cells behind the molecular defect 159
Oxidative stress modulates heme levels and induces peroxiredoxin-2 in β thalassemic erythropoiesis as novel cytoprotective response 158
Folding pathway of the pyridoxal 5'-phosphate C-S lyase MalY from Escherichia coli 157
Aromatic amino acid methyl ester analogs form quinonoidal species with Dopa decarboxylase 155
Behavior of fluorinated analogs of L-(3,4-dihydroxyphenyl)alanine and L-threo-(3,4-dihydroxyphenyl)serine as substrates for Dopa decarboxylase 151
Phosphorylation of pyridoxal 5'-phosphate enzymes: an intriguing and neglected topic 148
New variants of AADC deficiency expand the knowledge of enzymatic phenotypes 148
Cysteine 180 Is a Redox Sensor Modulating the Activity of Human Pyridoxal 5'-Phosphate Histidine Decarboxylase 147
Mutation of residues in the coenzyme binding pocket of Dopa decarboxylase: effects on catalytic properties 143
Deoxygenation affects tyrosine phosphoproteome of red cell membrane from patients with sickle cell disease 142
Resveratrol accelerates erythroid maturation by activation of FoxO3 and ameliorates anemia in beta-thalassemic mice 140
New insights emerging from recent investigations on human group II pyridoxal 5'-phosphate Decarboxylases 140
Site-directed mutagenesis provides insight into racemization of alanine catlayzed by "Treponema denticola" cystalysin 139
Aromatic amino acid decarboxylase deficiency: Molecular and metabolic basis and therapeutic outlook 139
Compound heterozygosis in AADC deficiency: A complex phenotype dissected through comparison among heterodimeric and homodimeric AADC proteins 137
Dopa decarboxylase 134
Oxidative stress modulates heme synthesis and induces peroxiredoxin-2 as a novel cytoprotective response in β-thalassemic erythropoiesis 132
The novel role of peroxiredoxin-2 in red cell membrane protein homeostasis and senescence. 131
Oxygen reactivity with pyridoxal 5'-phosphate enzymes: biochemical implications and functional relevance 131
Pharmacological inhibition of calpain-1 prevents red cell dehydration and reduces Gardos channel activity in a mouse model of sickle cell disease 129
Spectroscopic and kinetic analyses reveal the pyridoxal 5'-phosphate binding mode and the catalytic features of Treponema denticola cystalysin 124
A novel compound heterozygous genotype associated with aromatic amino acid decarboxylase deficiency: Clinical aspects and biochemical studies 121
Tyrosine phosphorylation modulates peroxiredoxin-2 activity in normal and diseased red cells 121
Lysine 238 is an essential residue for alfa, beta- elimination catalyzed by "Treponema denticola" cystalysin 118
Insights into the mechanism of oxidative deamination catalyzed by Dopa decarboxylase 118
Probing the role of Tyr 64 of Treponema denticola cystalysin by site-directed mutagenesis and kinetic studies 117
Mutation of cysteine 111 in dopa decarboxylase leads to active site perturbation 117
Dopa decarboxylase 116
Aromatic l-amino acid decarboxylase deficiency: a patient-derived neuronal model for precision therapies 115
Mechanism-based inactivation of dopa decarboxylase by serotonin 114
Structural versatility of bovine ribonuclease A: distinct conformers of trimeric and tetrameric aggregates of the enzyme 114
Does the aromatic L-amino acid decarboxylase contribute to thyronamine biosynthesis? 112
Heterozygosis in aromatic amino acid decarboxylase deficiency: Evidence for a positive interallelic complementation between R347Q and R358H mutations 112
Peroxiredoxin-2 (Prx2) expression is increased in beta thalassemic mouse red cells but displaced from the membrane as marker of oxidative stress 110
Treponema denticola cystalysin catalyzes beta-desulfination of L-cysteine sulfinic acid and beta-decarboxylation of L-aspartate and oxalacetate 107
Parkinson's Disease: recent updates in the identification of human dopa decarboxylase inhibitors 107
Reaction specificity of native and nicked 3,4-dihydroxyphenylalanine decarboxylase 107
Ornithine and glutamate decarboxylases catalyse an oxidative deamination of their alpha-methyl substrates 105
Reactions of human liver peroxisomal alanine:glyoxylate aminotransferase with beta-chloro-L-alanine and L-cysteine: spectroscopic and kinetic analysis 105
Mutation of tyrosine 332 to phenylalanine converts Dopa decarboxylase into a decarboxylation-dependent oxidative deaminase 101
Reaction of Dopa decarboxylase with alfa-methylDopa leads to an oxidative deamination producing 3,4-dihydroxyphenylacetone, an active-site directed affinity label 100
The activity on double-stranded RNA of aggregates of Ribonuclease A higher than dimers increases as a function of the size of the aggregates 100
Protective effect of epigallocatechin-3-gallate (EGCG) in diseases with uncontrolled immune activation: Could such a scenario be helpful to counteract COVID-19? 100
Treponema denticola cystalysin exhibits a significant alanine racemase activity accompanied by transamination: mechanistic implications 98
Compound heterozygosis in AADC deficiency and its complex phenotype in terms of AADC protein population 97
Human peroxiredoxins 1 and 2 and their interacting protein partners; through structure toward functions of biological complexes 97
Human aromatic amino acid decarboxylase is an asymmetric and flexible enzyme: implication in AADC deficiency 96
Autism spectrum disorder and GABA levels in children with succinic semialdehyde dehydrogenase deficiency 94
An attenuated, adult case of AADC deficiency demonstrated by protein characterization 94
Mapping of human autoantibody epitopes on aromatic l-amino acid decarboxylase 93
The novel P330L pathogenic variant of aromatic amino acid decarboxylase maps on the catalytic flexible loop underlying its crucial role 93
Multiple roles of the active site lysine of Dopa decarboxylase 90
Structural insights into the heme pocket and oligomeric state of non-symbiotic hemoglobins from Arabidopsis thaliana 89
The CRISPR-Cas9 knockout DDC SH-SY5Y in vitro model for AADC deficiency provides insight into the pathogenicity of R347Q and L353P variants: a cross-sectional structural and functional analysis 88
Reaction and substrate specificity of pig kidney Dopa decarboxylase under aerobic and anaerobic conditions 87
Exome sequencing data screening to identify undiagnosed Aromatic l-amino acid decarboxylase deficiency in neurodevelopmental disorders 83
Functional Characterization of a Spectrum of Genetic Variants in a Family with Succinic Semialdehyde Dehydrogenase Deficiency 83
Prevalence of DDC genotypes in patients with aromatic L-amino acid decarboxylase (AADC) deficiency and in silico prediction of structural protein changes 82
A crucial active site network of titratable residues guides catalysis and NAD+ binding in human succinic semialdehyde dehydrogenase 80
Clinical and molecular outcomes from the 5-Year natural history study of SSADH Deficiency, a model metabolic neurodevelopmental disorder 80
Mammalian Dopa decarboxylase: structure, catalytic activity and inhibition 78
Human DOPA decarboxylase: catalysis and involvement in pharmacologica treatments of Parkinson's disease and aromatic amino acid decarboxylase deficiency 78
Case report: Childhood epilepsy and borderline intellectual functioning hiding an AADC deficiency disorder associated with compound heterozygous DDC gene pathogenic variants 77
Consensus guidelines for the diagnosis and management of succinic semialdehyde dehydrogenase deficiency 74
Mild/moderate phenotypes in AADC deficiency: Focus on the aromatic amino acid decarboxylase protein 73
Mitapivat metabolically reprograms human beta-thalassemic erythroblasts, increasing their responsiveness to oxidation 73
Active site serine-193 modulates activity of human aromatic amino acid decarboxylase 73
Succinic semialdehyde dehydrogenase deficiency: in vitro and in silico characterization of a novel pathogenic missense variant and analysis of the mutational spectrum of ALDH5A1 66
Central Dysmyelination in SSADH-Deficient Humans and Mice 59
Phenotypic correlates of structural and functional protein impairments resultant from ALDH5A1 variants 57
The presence and severity of epilepsy coincide with reduced GABA and cortical excitatory markers in SSADH deficiency 55
REVEAL-CP: Selective Screening of Pediatric Patients for Aromatic L-Amino Acid Decarboxylase Deficiency with a Guthrie Card and In Silico Structural Modeling of One Index Case 52
Navigating the rare neurotransmitter disease diagnosis: insights from patients and health care professionals 51
The integrated approach to inherited disorders in neurotransmitters from molecules to systems 44
α-Hydrazino Acids Inhibit Pyridoxal Phosphate-Dependent Decarboxylases via “Catalytically Correct” Ketoenamine Tautomers: A Special Motif for Chemical Biology and Drug Discovery? 43
Pharmacological Inhibition of Calpain-1 Prevents Red Cell Dehydration and Reduces Gardos Channel Activity in a Mouse Model of Sickle Cell Disease. Identification of Druggable Protease Target 41
Reaction of dopa decarboxylase with L-aromatic amino acids under aerobic and anaerobic conditions 41
Gene replacement therapies for inherited disorders of neurotransmission: Current progress in succinic semialdehyde dehydrogenase deficiency 40
Green tea polyphenols: novel irreversible inhibitors of dopa decarboxylase 36
PEROXIREDOXIN-2 (Prx2) MEMBRANE DISPLACEMENT IS A NEW ADDITIONAL FACTOR to the SEVERITY of beta-THALASSEMIC MOUSE RED CELL MEMBRANE DAMAGE 35
Consensus Guideline for the Diagnosis and Treatment of Tyrosine Hydroxylase (TH) Deficiency 33
Neurotransmitters … it is all about communication! 32
The neuropsychological profile of SSADH deficiency, a neurotransmitter disorder of GABA metabolism 32
Totale 11.141
Categoria #
all - tutte 38.091
article - articoli 35.648
book - libri 0
conference - conferenze 1.398
curatela - curatele 0
other - altro 393
patent - brevetti 0
selected - selezionate 0
volume - volumi 652
Totale 76.182


Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2020/2021271 0 0 0 0 0 69 9 62 25 7 62 37
2021/2022749 47 241 126 30 19 16 5 24 25 22 45 149
2022/20231.052 84 99 110 196 80 253 20 49 112 9 21 19
2023/2024580 30 48 32 93 60 66 22 25 12 19 127 46
2024/20251.966 102 88 46 347 62 46 97 86 307 152 213 420
2025/20263.892 430 279 482 860 1.512 329 0 0 0 0 0 0
Totale 11.141