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Multifaceted superoxide dismutase 1 expression in amyotrophic lateral sclerosis patients:a rare occurrence?

Neural Regeneration Research 2025年第1期20卷 130-138页

作者： ilaria martinelli Jessica Mandrioli Andrea Ghezzi Elisabetta Zucchi Giulia Gianferrari Cecilia Simonini Francesco Cavallieri Franco Valzania Department of Neurosciences Azienda Ospedaliero Universitaria di ModenaModenaItaly Clinical and Experimental Medicine Ph.D.Program University of Modena and Reggio EmiliaModenaItaly Department of Biomedical Metabolic and Neural SciencesUniversity of Modena and Reggio EmiliaModenaItaly Neurology Unit Neuromotor&Rehabilitation DepartmentAzienda USL-IRCCS di Reggio EmiliaReggio EmiliaItaly

Amyotrophic lateral sclerosis(ALS)is a neuromuscular condition resulting from the progressive degeneration of motor neurons in the cortex,brainstem,and spinal *** the typical clinical phenotype of ALS involves both upper and lower motor neurons,human and animal studies over the years have highlighted the potential spread to other motor and non-motor regions,expanding the phenotype of *** superoxide dismutase 1(SOD1)mutations represent a minority of ALS cases,the SOD1 gene remains a milestone in ALS research as it represents the first genetic target for personalized *** numerous single case reports or case series exhibiting extramotor symptoms in patients with ALS mutations in SOD1(SOD1-ALS),no studies have comprehensively explored the full spectrum of extramotor neurological manifestations in this *** this narrative review,we analyze and discuss the available literature on extrapyramidal and non-motor features during *** multifaceted expression of SOD1 could deepen our understanding of the pathogenic mechanisms,pointing towards a multidisciplinary approach for affected patients in light of new therapeutic strategies for SOD1-ALS.

关键词： amyotrophic lateral sclerosis(ALS) autonomic extramotor genotype-phenotype multisystem involvement Parkinson’s disease sensory SOD1 superoxide dismutase 1 urinary vocal cord palsy

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The landscape of cognitive impairment in superoxide dismutase 1-amyotrophic lateral sclerosis

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Neural Regeneration Research 2023年第7期18卷 1427-1433页

作者： ilaria martinelli Elisabetta Zucchi Cecilia Simonini Giulia Gianferrari Giovanna Zamboni Marcello Pinti Jessica Mandrioli Clinical and Experimental Medicine PhD Program University of Modena and Reggio EmiliaModenaItaly Department of Neurosciences Azienda Ospedaliero-Universitaria di ModenaModenaItaly Department of Biomedical Metabolic and Neural SciencesUniversity of Modena and Reggio EmiliaModenaItaly Department of Life Sciences University of Modena and Reggio EmiliaModenaItaly

Although mutations in the superoxide dismutase 1 gene account for only a minority of total amyotrophic lateral sclerosis cases,the discovery of this gene has been crucial for amyotrophic lateral sclerosis *** the identification of superoxide dismutase 1 in 1993,the field of amyotrophic lateral sclerosis genetics has considerably widened,improving our understanding of the diverse pathogenic basis of amyotrophic lateral *** this review,we focus on cognitive impairment in superoxide dismutase 1-amyotrophic lateral sclerosis *** has mostly reported that cognition remains intact in superoxide dismutase 1-amyotrophic lateral sclerosis patients,but recent reports highlight frontal lobe function frailty in patients carrying different superoxide dismutase 1-amyotrophic lateral sclerosis *** thoroughly reviewed all the various mutations reported in the literature to contribute to a comprehensive database of superoxide dismutase 1-amyotrophic lateral sclerosis genotype-phenotype *** a resource could ultimately improve our mechanistic understanding of amyotrophic lateral sclerosis,enabling a more robust assessment of how the amyotrophic lateral sclerosis phenotype responds to different variants across genes,which is important for the therapeutic strategy targeting genetic *** in superoxide dismutase 1-amyotrophic lateral sclerosis deserves further longitudinal research since this peculiar frailty in patients with similar mutations can be conditioned by external factors,including environment and other unidentified agents including modifier genes.

关键词： amyotrophic lateral sclerosis cognitive impairment genotype-phenotype correlation superoxide dismutase 1

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Disentangling brain PrP^(C)proteoforms and their roles in physiology and disease

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Neural Regeneration Research 2024年第5期19卷 963-965页

作者： ilaria Vanni Nonno Romolo Department of Food Safety Nutrition and Veterinary Public HealthIstituto Superiore di SanitàRomeItaly

The cellular prion protein(PrPC),a cell surface glycoprotein of 209 amino acids,has been considerably studied over the decades mainly due to its critical involvement in transmissible spongiform encephalopathies,or prion ***,it is the misfolding and aggregation of PrPC into pathological assemblies-named PrPSc-that constitute prions,the agents causing these unusual neurodegenerative diseases affecting humans and animals(Prusiner,1982).Furthermore,increasing evidence support its relevance also in other neurodegenerative diseases(NDDs),such as Alzheimer’s and Parkinson’s diseases(Corbett et al.,2020).

关键词： diseases involvement glycoprotein

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Frequency stabilization of C-band semiconductor lasers through a SiN photonic integrated circuit

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Photonics Research 2024年第8期12卷 1619-1626页

作者： ALESSANDRO BRUGNONI ALI EMRE KAPLAN VALERIO VITALI KYLE BOTTRILL MICHELE RE PERIKLIS PETROPOULOS COSIMO LACAVA ilaria CRISTIANI Photonics Group Department of ElectricalComputer and Biomedical EngineeringUniversity of PaviaPavia 27100Italy Optoelectronics Research Centre University of SouthamptonSouthampton SO171BJUK Huawei Technologies Italia S.r.l Centro direzionale MilanoSegrate 20054Italy

Integrated semiconductor lasers represent essential building blocks for integrated optical components and circuits and their stability in frequency is fundamental for the development of numerous frontier applications and engineering *** dense optical circuits are considered,the stability of integrated laser sources can be impaired by the thermal cross-talk generated by the action of neighboring components,leading to a deterioration of the long-term system performance(on the scale of seconds).In this work we show the design and the experimental characterization of a silicon nitride photonic integrated circuit(PIC)that is able to frequency stabilize 16semiconductor lasers,simultaneously.A stabilized 50 GHz-spaced two-channel system is demonstrated through the detection of the related beating note and the stability of the resulting waveform is characterized via the use of artificially induced thermal cross-talk stimuli.

关键词： stabilization stability impaired

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Advancing immunosuppression in liver transplantation: A narrative review

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Hepatobiliary & Pancreatic Diseases International 2024年第5期23卷 441-448页

作者： Samuele Iesari Francesca Laura Nava ilaria Elena Zais Laurent Coubeau Mariano Ferraresso Evaldo Favi Jan Lerut General Surgery and Kidney Transplantation Fondazione IRCCS Ca’Granda Ospedale Maggiore Policlinico15 Via della Commenda20122 MilanItaly Institut de Recherche Expérimentale et Clinique Universitécatholique de Louvain10 Avenue Hippocrate1200 BrusselsBelgium Service de Chirurgie et Transplantation Abdominale Cliniques Universitaires Saint-Luc55 Avenue Hippocrate1200 BrusselsBelgium Department of Clinical Sciences and Community Health Universitàdegli Studi di Milano19 Via della Commenda20122 MilanItaly

Immunosuppression is essential to ensure recipient and graft survivals after liver transplantation(LT). However, our understanding and management of the immune system remain suboptimal. Current immunosuppressive therapy cannot selectively inhibit the graft-specific immune response and entails a significant risk of serious side effects, i.e., among others, de novo cancers, infections, cardiovascular events, renal failure, metabolic syndrome, and late graft fibrosis, with progressive loss of graft function. Pharmacological research, aimed to develop alternative immunosuppressive agents in LT, is behind other solidorgan transplantation subspecialties, and, therefore, the development of new compounds and strategies should get priority in LT. The research trajectories cover mechanisms to induce T-cell exhaustion, to inhibit co-stimulation, to mitigate non-antigen-specific inflammatory response, and, lastly, to minimize the development and action of donor-specific antibodies. Moreover, while cellular modulation techniques are complex, active research is underway to foster the action of T-regulatory cells, to induce tolerogenic dendritic cells, and to promote the function of B-regulatory cells. We herein discuss current lines of research in clinical immunosuppression, particularly focusing on possible applications in the LT setting.

关键词： Liver transplantation Immunosuppression Drug development Long-term results Clinical operational tolerance

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Does the provision of live black soldier fly and yellow mealworm larvae improve Muscovy duck welfare?

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Journal of Animal Science and Biotechnology 2024年第1期15卷 442-455页

作者： Marta Gariglio Sihem Dabbou Manuela Renna ilaria Biasato Sara Bellezza Oddon Marco Meneguz Raul Daniel Miazzo Stefania Bergagna Elena Colombino Elisabetta Macchi Achille Schiavone Department of Veterinary Sciences University of TurinLargo Paolo Braccini 2GrugliascoTorino(TO)10095Italy Center Agriculture Food Environment(C3A) University of TrentoVia Edmund Mach 1San Michele All’AdigeTrento(TN)38098Italy Department of Agricultural Forest and Food SciencesUniversity of TurinLargo Paolo Braccini 2GrugliascoTorino(TO)10095Italy BEF Biosystems s.r.l Via Tancredi Canonico 18/CTorino(TO)10156Italy Unidad de Investigacion Aviar Produccion AvicolaFac.Agr.y Vet.Universidad Nacional de Rio CuartoCordobaArgentina Veterinary Medical Research Institute for Piemonte Liguria and Valle d’AostaVia Bologna 148Torino(TO)10154Italy

Background The provision of environmental enrichments to Muscovy ducks could reduce the expression of the aggressive *** aim of the present study was to evaluate the effects of black soldier fly(BSF)and yellow mealworm(YM)live larva provision on Muscovy duck performance,excreta corticosterone metabolites(ECM),behavior,and blood *** A total of 1263-day-old female Muscovy ducklings were allotted to 18 pens(6 replicates/treatment,7 birds/pen)and assigned to 3 experimental treatments:a control group fed commercial feed,and two experimental treatments fed commercial feed plus the 5%(based on the expected daily feed intake,as fed basis)of BSF and YM live larvae(BSF and YM groups,respectively).A two-phase feeding program was applied:starter(from 3 to 31 days of age)and grower-finisher(from 32 to 55 days of age).The live weight,average daily gain,average daily feed intake,and feed conversion ratio were *** consumption times were collected,and video recordings were performed during 3 periods(P)each day:the hour before(P1),during(P2),and after(P3)the larva *** were evaluated at 3,31,and ***,the total red and white blood cell counts,serum proteins,lipids,and liver and renal function serum enzymes were evaluated on 12 birds/*** The experimental treatment did not affect the growth performance of the birds(P>0.05).Larva consumption times were always similar between the two insect species,except at 14–18 days of age,were BSF larvae were consumed faster than YM larvae(PP3;P<0.05).Finally,the provision of live BSF and YM larvae significantly reduced the ECM at 55 days of age and the heterophil to lymphocyte ratio(P<0.05).Conclusions Live BSF and YM larva supplementatio

关键词： Behavior Cairina moschata Environmental enrichment Hermetia illucens Poultry Tenebrio molitor Welfare

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Asymptotic Formulae for Multivariate Kantorovich Type Generalized Sampling Series

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Acta Mathematica Sinica,English Series 2011年第7期27卷 1247-1258页

作者： Carlo BARDARO ilaria MANTELLINI Dipartimento di Matematica e Informatica Universitd di Perugia Via Vanvitelli 1 06123 Perugia Italy

In this paper an asymptotic formula of Voronovskaja type for a multivariate extension of the Kantorovich generalized sampling series is given. Moreover a quantitative version in terms of some moduli of smoothness is established. Finally some particular examples of kernels are discussed, as the Bochner-Riesz kernel and the multivariate splines.

关键词： Voronovskaja-type formula moments multivariate Kantorovich generalized sampling series Peetre K-functional

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Spinterface:A new platform for spintronics

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Nano Materials Science 2019年第3期1卷 149-155页

作者： ilaria Bergenti Valentin Dediu Institute of Nanostructured Materials ISMN National Research Council CNR40129BolognaItaly CNR-ISMN Bologna Via P.Gobetti 10140129BolognaItaly

Since its discovery in early 2000’s Molecular Spintronics has developed in an established and fructuous research field,achieving a number of outstanding results and unveiling unusual spintronic *** the most mysterious device element,the interface,in molecular spintronics has on contrary received and enormous attention and even gained a special nickname–the *** on significant efforts of many research groups worldwide it has been established its critical role in defining the main functionalities of molecular spintronic *** the spinterface was found to control the properties of the both components constituting the interface,not only those of the molecular layer but surprisingly also those of the magnetic *** paper aims to overview the most striking spinterface properties and to highlight the possibilities to promote new device paradigms based on interfacial modulation.

关键词： Molecular spintronics Ferromagnetism Spin polarization Hybridization organic semiconductors

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The new K_V3.4 inhibitor BDS-I[1–8] as a potential pharmacological opportunity in Alzheimer’s disease therapy

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Neural Regeneration Research 2020年第7期15卷 1255-1256页

作者： ilaria Piccialli Roselia Ciccone Anna Pannaccione Division of Pharmacology Department of NeuroscienceReproductive and Dentistry Sciences School of Medicine Federico II University of Naples

Alzheimer's disease(AD)is the most common neurodegenerative disorder and the first cause of dementia in the elderly,with no treatment able to prevent or to block disease *** is characterized by memory impairment and cognitive dysfunction,followed in the late phases of the disease by severe neurodegeneration and neuronal *** amyloid-β(Aβ)peptide,generated upon the processing of the amyloid precursor protein,is considered the main initiator of AD ***,Aβpeptides,which aggregate and accumulate to form extracellular plaques and intraneuronal deposits.

关键词： inhibitor BDS-I[1-8] BDS Alzheimer

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Isoenergetic-practical and semi-purified diets for protein requirement determination in Hermetia illucens larvae:consequences on life history traits

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Journal of Animal Science and Biotechnology 2022年第3期13卷 897-906页

作者： Sara Bellezza Oddon ilaria Biasato Laura Gasco Department of Agricultural Forest and Food SciencesUniversity of TurinLargo P.Braccini 210095 GrugliascoTOItaly

Background:Black soldier fly(BSF)is one of the most promising species for the intensive breeding of insects given its adaptability and its efficiency in the conversion of *** maximize the production and use waste as substrates,it is essential to determine the larvae nutritional *** study aims to evaluate the effects of5 practical,semi-purified and isoenergetic diets(PSPID)with increasing protein levels(10%,CP10;14%,CP14;16%,CP16;19%,CP19)on BSF life history traits.A total of 2000 six-day-old larvae were weighed and divided into groups of 100(4 replicates/treatment[PSPID and Gainesville diet(GA)used as environmental control]).In larva-prepupa stage,sampling was carried out on 30 individuals/replicate,while in prepupa-adult stage on ***:In the whole larval stage,the CP16 treatment performed better when compared to the other ***18 days old,the CP14 treatment showed a higher weight than the CP19(P<0.01),while the CP10 and CP16weights were *** the contrary,the CP10 prepupae weight was higher than the CP19(P<0.01).The CP10 and CP14 pupae performed better in terms of weight when compared to the others(P<0.01).The dietary treatments did not affect the adult *** sex significantly influenced both the exuvia weight,which was the greatest in females(P<0.001),and the fly lifespan,longest in males(P<0.05).Fly lifespan was also influenced by the interaction between treatment and sex,with the CP19 females showing a longer life than the others(P<0.05).Conclusions:In conclusion,the optimal protein level considering the whole larval stage is 16%on dry matter(DM).At 18 days old,looking at the size intended for the meal production,it can be considered 14%on *** result obtained on adult emergence in PSPID may not be attributable to the protein *** researches on macronutrients requirements determination have to be conducted to evaluate the substrates composition effects on BSF life history traits.

关键词： Hermetia illucens Life history traits Nutritional requirements Semi-purified

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