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Therapeutic advances in neural regeneration for Huntington’s disease

Neural Regeneration Research 2024年第9期19卷 1991-1997页

作者： francesco d’Egidio Vanessa Castelli Giorgia lombardozzi Fabrizio Ammannito Annamaria Cimini Michele d’Angelo department of life Health and Environmental SciencesUniversity of L’AquilaL’AquilaItaly

Huntington’s disease is a neurodegenerative disease caused by the expansion mutation of a cytosine-adenine-guanine triplet in the exon 1 of the HTT gene which is responsible for the production of the huntingtin (Htt) protein. In physiological conditions, Htt is involved in many cellular processes such as cell signaling, transcriptional regulation, energy metabolism regulation, dNA maintenance, axonal trafficking, and antiapoptotic activity. When the genetic alteration is present, the production of a mutant version of Htt (mHtt) occurs, which is characterized by a plethora of pathogenic activities that, finally, lead to cell death. Among all the cells in which mHtt exerts its dangerous activity, the GABAergic Medium Spiny Neurons seem to be the most affected by the mHtt-induced excitotoxicity both in the cortex and in the striatum. However, as the neurodegeneration proceeds ahead the neuronal loss grows also in other brain areas such as the cerebellum, hypothalamus, thalamus, subthalamic nucleus, globus pallidus, and substantia nigra, determining the variety of symptoms that characterize Huntington’s disease. From a clinical point of view, Huntington’s disease is characterized by a wide spectrum of symptoms spanning from motor impairment to cognitive disorders and dementia. Huntington’s disease shows a prevalence of around 3.92 cases every 100,000 worldwide and an incidence of 0.48 new cases every 100,000/year. To date, there is no available cure for Huntington’s disease. Several treatments have been developed so far, aiming to reduce the severity of one or more symptoms to slow down the inexorable decline caused by the disease. In this context, the search for reliable strategies to target the different aspects of Huntington’s disease become of the utmost interest. In recent years, a variety of studies demonstrated the detrimental role of neuronal loss in Huntington’s disease condition highlighting how the replacement of lost cells would be a reasonable strategy to o

关键词： cell therapy exosomes extracellular vesicles Huntingtin Huntington’s disease medium spiny neurons neurodegenerative disease neurogenesis neuronal loss stem cells

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Highly sensitive ratiometric fluorescent fiber matrices for oxygen sensing with micrometer spatial resolution

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Bio-design and Manufacturing 2024年第3期7卷 292-306页

作者： Giuliana Grasso Valentina Onesto Stefania Forciniti Eliana d’Amone francesco Colella lara Pierantoni Valeria Famà Giuseppe Gigli Rui l.Reis J.Miguel Oliveira loretta l.d.l Mercato nstitute of Nanotechnology National Research Council(CNR-NANOTEC)c/o Campus Ecoteknevia Monteroni73100 LecceItaly department of Mathematics and Physics“Ennio de Giorgi” University of Salentoc/o Campus Ecoteknevia Monteroni73100 LecceItaly 3B’s Research Group I3Bs–Research Institute on BiomaterialsBiodegradables and BiomimeticsHeadquarters of the European Institute of Excellence on Tissue Engineering and Regenerative MedicineUniversity of MinhoAveParkZona Industrial da Gandra4805-017 BarcoGuimarãesPortugal ICVS/3B’s-PT Government Associate laboratory 4806-909 Braga/GuimarãesPortugal department of Experimental Medicine University of Salentoc/o Campus Ecoteknevia Monteroni73100 LecceItaly

Oxygen(O_(2))-sensing matrices are promising tools for the live monitoring of extracellular O_(2) consumption levels in long-term cell *** this study,ratiometric O_(2)-sensing membranes were prepared by electrospinning,an easy,low-cost,scalable,and robust method for fabricating ***(ε-caprolactone)and poly(dimethyl)siloxane polymers were blended with tris(4,7-diphenyl-1,10-phenanthroline)ruthenium(II)dichloride,which was used as the O_(2)-sensing probe,and rhodamine B isothiocyanate,which was used as the reference *** functionalized scaffolds were morphologically characterized by scanning electron microscopy,and their physicochemical profiles were obtained by Fourier transform infrared spectroscopy,thermogravimetric analysis,and water contact angle *** sensing capabilities were investigated by confocal laser scanning microscopy,performing photobleaching,reversibility,and calibration curve studies toward different dissolved O_(2)(dO)*** sensing nanofibers showed a high response to changes in dO concentrations in the physiological-pathological range from 0.5%to 20%and good stability under ratiometric *** addition,the sensing systems were highly biocompatible for cell growth promoting adhesiveness and growth of three cancer cell lines,namely metastatic melanoma cell line SK-MEl2,breast cancer cell line MCF-7,and pancreatic ductal adenocarcinoma cell line Panc-1,thus recreating a suitable biological environment in *** O_(2)-sensing biomaterials can potentially measure alterations in cell metabolism caused by changes in ambient O_(2)content during drug testing/validation and tissue regeneration processes.

关键词： Electrospinning Ruthenium(II)dichloride Oxygen sensors Ratiometric imaging Fluorescence

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Perfluorinated iodine alkanes promote the differentiation of mouse embryonic stem cells by regulating estrogen receptor signaling

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Journal of Environmental Sciences 2024年第3期137卷 443-454页

作者： Zhihua Ren Xiaoxi Yang Tingting Ku Qian S.liu Jiefeng liang Qunfang Zhou francesco Faiola Guibin Jiang State Key laboratory of Environmental Chemistry and Ecotoxicology Research Center for Eco-Environmental SciencesChinese Academy of SciencesBeijing 100085China College of Resources and Environment University of Chinese Academy of SciencesBeijing 100049China College of Environment and Resource Research Center of Environment and HealthShanxi UniversityTaiyuan 030006China College of Sino-danish University of Chinese Academy of SciencesBeijing 100049China

Investigating the development toxicity of perfuorinated iodine alkanes(PFIs)is critical,given their estrogenic effects through binding with estrogen receptors(ERs).In the present study,two PFIs,including dodecafuoro-1,6-diiodohexane(PFHx dI)and tridecafuorohexyl iodide(PFHx I),with binding preference to ERαand ERβ,respectively,were selected to evaluate their effects on proliferation and differentiation of the mouse embryonic stem cells(m ESCs).The results revealed that,similar to E_(2),50μmol/l PFHx dI accelerated the cell proliferation of the m *** PFI stimulation at the exposure concentrations of 2–50μmol/l promoted the differentiation of the m ESCs as characterized by the upregulation of differentiation-related biomarkers(i.e.,Otx2 and dnmt3β)and downregulation of pluripotency genes(i.e.,Oct4,Nanog,Sox2,Prdm14 and Rex1).Comparatively,PFHx dI exhibited higher induction effect on the differentiation of the m ESCs than did PFHx *** tests on ER signaling indicated that both PFI compounds induced exposure concentration-dependent expressions of ER signaling-related biomarkers(i.e.,ERα,ERβand Caveolin-1)in the m ESCs,and the downstream ER responsive genes(i.e.,c-fos,c-myc and c-jun)well responded to PFHx I *** role of ER in PFI-induced effects on the m ESCs was further validated by the antagonistic experiments using an ER inhibitor(ICI).The findings demonstrated that PFIs triggered ER signaling,and perturbed the differentiation program of the m ESCs,causing the potential health risk during early stage of development.

关键词： Perfuorinated iodine alkanes(PFIs) Embryonic stem cells(ESCs) developmental toxicity cell differentiation Estrogen receptor-related signaling

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PlCG2 Mutation in a Patient Presenting with Type 2 Autoimmune Hepatitis

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Journal of Clinical and Translational Hepatology 2024年第7期12卷 685-687页

作者： Antoine Gardin léa Gaigne Aude Magérus Frédéric Rieux-laucat Emmanuel Jacquemin Pediatric Hepatology and.liver Transplantation Unit National Reference Centre for Inflammatory Diseases of the Bile Ducts and Autoimmune HepatitisFILFOIEERN RARE LIVERBicêtre HospitalAssistance Publique–Hôpitaux de ParisUniversity Paris-SaclayLe Kremlin-BicêtreFrance INSERM UMR 1193 HepatinovParis-Saclay UniversityOrsayFrance laboratory of Immunogenetics of Pediatric Autoimmune diseases INSERM UMR 1163Imagine InstituteParis CitéUniversityParisFrance

The combination of defective immune tolerance and external triggers is thought to underlie the pathophysiology of autoimmune hepatitis.1,2 Some polymorphisms associated with susceptibility to autoimmune hepatitis have been reported,and some cases have been described in patients with monogenic primary immune deficiencies or systemic diseases.

关键词： autoimmune Autoimmune immune

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Minimally invasive surgical therapies(MISTs)for lower urinary tract symptoms(lUTS):promise or panacea?

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Asian Journal of Andrology 2024年第2期26卷 135-143页

作者： Gian Maria Busetto Andrea Checchia Marco Recchia Edoardo Tocci Ugo G Falagario Gennaro Annunziata Pasquale Annese Nicola d'Altilia Vito Mancini Matteo Ferro Felice Crocetto Octavian Sabin Tataru luca di Gianfrancesco Angelo Porreca francesco d.l giud.ce Ettore de Berardinis Carlo Bettocchi luigi Cormio Giuseppe Carrieri University of Foggia Foggia 71122Italy department of Urology and Renal Transplantation Policlinico of FoggiaFoggia 71122Italy Urology Unit "G.Tatarlla"HospitalCerignola 71042Italy Urology Unit European Institute of Oncology(IE0)IRCcSMilan 20141Italy University of Naples Federico Il Naples 80126Italy G.E.Palade University of Medicine PharmacyScienceand Technology of Targu MuresTargu Mures 540142Romania department of Oncological Urology Veneto Institute of Oncology(IOv)IRccSPadua 35128Italy department of Maternal-Infant and Urological Sciences Sapienza Rome UniversityRoma 00161Italy Urology Unit "L.Bonomo"HospitalAndria 76123Italy

The increasing importance of treatment of lower urinary tract symptoms(lUTS),while avoiding side effects and maintaining sexual function,has allowed for the development of minimally invasive surgical therapies(MiSTs).Recently,the European Association of Urology guid.lines reported a paradigm shift from the management of benign prostatic hyperplasia(BPH)to the management of nonneurogenic male *** aim of the present review was to evaluate the efficacy and safety of the most commonly used MisTs:ablative techniques such as aquablation,prostatic artery embolization,water vapor energy,and transperineal prostate laser ablation,and nonablative techniques such as prostatic urethral lift and temporarily implanted nitinol device(iTINd).MiSTs are becoming a new promise,even if clinical trials with longer follow-up are still *** of them are still under investigation and,to date,only a few options have been given as a recommendation for *** cannot be considered as standard of care and are not suitable for all *** and disadvantages should be underlined,without forgetting our objective:treatment of lUTS and re-treatment avoidance.

关键词： aquablation iTINd prostatic artery embolization prostatic urethral lift transperineal prostate laser ablation water vapor energy

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Greatly enhanced corrosion/wear resistances of epoxy coating for Mg alloy through a synergistic effect between functionalized graphene and insulated blocking layer

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Journal of Magnesium and Alloys 2024年第1期12卷 332-344页

作者： Z.Y.Xue X.J.li J.H.Chu M.M.li d.N.Zou l.B.Tong School of Metallurgical Engineering Xi’an University of Architecture and TechnologyXi’an 710055China State Key laboratory of Advanced design and Manufacturing for Vehicle Body Hunan UniversityChangsha 410082China

The poor corrosion and wear resistances of Mg alloys seriously limit their potential applications in various *** conventional epoxy coating easily forms many intrinsic defects during the solidification process,which cannot provide sufficient *** the current study,we design a double-layer epoxy composite coating on Mg alloy with enhanced anti-corrosion/wear properties,via the spin-assisted assembly *** outer layer is functionalized graphene(FG)in waterborne epoxy resin(WEP)and the inner layer is ce-based conversion(ce)*** FG sheets can be homogeneously dispersed within the epoxy matrix to fill the intrinsic defects and improve the barrier *** ce film connects the outer layer with the substrate,showing the transition *** corrosion rate of ce/WEP/FG composite coating is 2131 times lower than that of bare Mg alloy,and the wear rate is decreased by~90%.The improved corrosion resistance is attributed to the labyrinth effect(hindering the penetration of corrosive medium)and the obstruction of galvanic coupling *** synergistic effect derived from the FG sheet and blocking layer exhibits great potential in realizing the improvement of multi-functional integration,which will open up a new avenue for the development of novel composite protection coatings of Mg alloys.

关键词： Mg alloy Functionalized graphene Epoxy coating Corrosion/wear resistance Blocking layer

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Hydroxysafflor Yellow A Inhibits Pyroptosis and Protecting HUVECs from OGd/R via NlRP3/Caspase-1/GSdMd Pathway

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Chinese Journal of Integrative Medicine 2024年第11期30卷 1027-1034页

作者： GUO Fan HAN Xiao YOU Yue XU Shu-juan ZHANG Ye-hao CHEN Yuan-yuan XIN Gao-jie lIU Zi-xin REN Jun-guo CAO ce lI ling-mei FU Jian-hua Institute of Basic Medical Sciences of Xiyuan Hospital China Academy of Chinese Medical Sciences Beijing Key laboratory of Chinese Materia Pharmacology department of central laboratory Kunshan Hospital of Chinese Medicine

Objective: To observe the protective effect and mechanism of hydroxyl safflower yellow A(HSYA)from myocardial ischemia-reperfusion injury on human umbilical vein endothelial cells(HUVECs). Methods:HUVECs were treated with oxygen-glucose deprivation reperfusion(OGd/R) to simulate the ischemia reperfusion mod.l, and cell counting kit-8 was used to detect the protective effect of different concentrations(1.25–160 μmol/l)of HSYA on HUVECs after OGd/R. HSYA 80 μmol/l was used for follow-up experiments. The contents of inflammatory cytokines interleukin(Il)-18, Il-1β, monocyte chemotactic protein 1(MCP-1), tumor necrosis factor α(TNF-α) and Il-6 before and after administration were measured by enzyme-linked immunosorbent assay. The protein expressions of toll-like receptor, NOd.like receptor containing pyrin domain 3(NlRP3), gasdermin d(GSdMd) and GSdMd-N-terminal domain(GSdMd-N) before and after administration were detected by Western blot. NlRP3 inflammasome inhibitor cytokine release inhibitory drug 3 sodium salt(CRId3 sodium salt, also known as MCC950) and agonist were added, and the changes of NlRP3, cysteine-aspartic acid protease 1(Caspase-1), GSdMd and GSdMd-N protein expressions were detected by Western blot. Results: HSYA inhibited OGd/R-induced inflammation and significantly decreased the contents of inflammatory cytokines Il-18,Il-1β, MCP-1, TNF-α and Il-6(PlRP3/Caspase-1/GSdMd pathway, HSYA can reduce the occurrence of pyroptosis after OGd/R and reduce the expression of NlRP3,Caspase-1, GSdMd and GSdMd-N proteins(Plusions: The protective effect of HSYA on HUVECs after OGd/R is related to down-regulating the expression of NlRP3 inflammasome and inhibiting pyroptosis.

关键词： Chinese medicine NlRP3 inflammasome human umbilical vein endothelial cells hydroxysafflor yellow A myocardial reperfusion injury oxygen-glucose deprivation reperfusion pyroptosis

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Corneal collagen cross‑linking epithelium‑on ***‑off:a systematic review and meta‑analysis

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Eye and Vision 2021年第6期8卷 338-352页

作者： francesco d’Oria Antonio Palazón Jorge l.Alio Vissum Innovation c/Cabañal103016 AlicanteSpain division of Ophthalmology Universidad Miguel HernándezAlicanteSpain Section of Ophthalmology Department of Basic Medical ScienceNeuroscience and Sense OrgansUniversity of BariBariItaly department of Clinical Medicine Miguel Hernández UniversitySan Juan de AlicanteSpain

Background:The purpose of the study was to determine the advantages and disadvantages of epi-on corneal crosslinking(CXl)techniques compared with standard epi-off ***:We searched MEdlINE and EMBASE for randomized controlled trials(RCTs)and non-randomized studies of interventions(NRSIs)and we evaluated the selected papers according to the Cochrane risk of bias *** considered,as primary outcomes,average Kmax flattening,changes in uncorrected and corrected distance visual acuity(UdVA and CdVA);as secondary outcomes,we considered changes in pachymetry values and endothelial cell density(ECd).We also investigated adverse events related to the treatments and treatment ***-analysis was conducted with a fixed or random-effects mod.l using weighted mean difference(Md)with 95%confidence interval(CI)as the effect ***:A total of 15 studies were included and among these 15 trials,9 were RCTs and 6 were NRSIs,but only 4 studies showed no high risk of bias and were included in this *** analysis revealed significant postoperative differences in CdVA(Md=0.07;95%CI 0.04 to 0.10;Pd no significative differences in UdVA,Kmax,central corneal thickness(CCT)and ECd(P>0.05).Epi-on CXl protocol was found to be significantly less prompt to have risks of d.lay in epithelial healing(P=0.035)and persistent stromal haze(P=0.026).Conclusion:Epi-on CXl is as effective as epi-off *** for a higher significant improvement in CdVA with current epi-on protocols,our meta-analysis demonstrates that epi-on and epi-off CXl have comparable effects on visual,topographic,pachymetric,and endothelial ***-on CXl has clinical advantages in terms of comfort and avoidance of complications as it reduces the risk of developing d.lay in epithelial healing and persistent stromal haze.

关键词： Corneal collagen cross-linking Keratoconus Transepithelial CXl Epithelium-off CXl Epithelium-on CXl Iontophoresis

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Reply to the letter to the editor

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Eye and Vision 2021年第1期8卷 462-463页

作者： Jorge l.Alio francesco Versaci francesco d’Oria Vissum Miranza AlicanteSpain division of Ophthalmology Universidad Miguel HernándezAlicanteSpain R&d department Costruzione Strumenti Oftalmici(CSO)FlorenceItaly Section of Ophthalmology Department of Basic Medical ScienceNeuroscience and Sense OrgansUniversity of BariBariItaly

We appreciate the interest of Miguel Faria Ribeiro in our paper[1],as well as the comments made to *** interest of the topic is sound,as nowadays an enormous amount of new so-called“premium lenses”are appearing in the market,with different optical profiles which obviously lead to different light distributions and different quality of retinal *** is why our interest in this topic is longstanding and we have published extensive summaries about the outcomes and complications of multifocal lenses over the last years[2,3].

关键词： editor letter enormous

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Retinal image quality with multifocal,EdoF,and accommodative intraocular lenses as studied by pyramid.l aberrometry

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Eye and Vision 2021年第6期8卷 374-384页

作者： Jorge l.Alio francesco d’Oria Francesca Toto Joan Balgos Antonio Palazon francesco Versaci Jorge l.Alio d.l Barrio Vissum Innovation AlicanteSpain division of Ophthalmology Universidad Miguel HernándezVissum Miranzac/Cabañal103016 AlicanteSpain Section of Ophthalmology Department of Basic Medical ScienceNeuroscience and Sense OrgansUniversity of BariBariItaly department of Clinical Medicine Miguel Hernández UniversitySan Juan de AlicanteSpain R&d department Costruzione Strumenti Oftalmici(CSO)FlorenceItaly

Background:To study and compare the clinical optical image quality following implantation with different premium IOls by analysing the point spread function(PSF)Strehl ratio using a pyramid.l wavefront sensor(PWS)-based ***:This study included 194 eyes implanted with:(a)19 AcrySof SA60AT(control group);(b)19 Miniwell;(c)24 lENTIS Mplus lS-313 MF30;(d)33 lENTIS Mplus lS-313 MF15;(e)17 Akkolens lumina;(f)31 AT lISA Tri 839MP;(g)20 Precizon Presbyopic;(h)20 AcrySof IQ PanOptix;(i)11 Tecnis *** outcome measures were PSF Strehl ratio,PSF Strehl ratio excluding second-order aberrations(PSFw2),total root mean square(RMS),low-order aberration(lOA)and high-order aberration(HOA)RMS measured by PWS ***:AT lISA Tri had the highest PSFw2 Strehl ratio at both 3.0-and 4.0-mm pupil size(0.52±0.14 and 0.31±0.10;Pllowed by SA60AT(0.41±0.11 and 0.28±0.07)and PanOptix(0.4±0.07 and 0.26±0.04).AT lISA Tri was found to provide a significantly better retinal image quality than PanOptix at both 3.0 mm(Pd 4.0 mm(P=0.004).Mplus MF15 was found to be significantly better than Mplus MF30 at both 3.0 mm(Pd 4.0 mm(P=0.002).Total RMS,lOA RMS,HOA RMS,PSF Strehl ratio and PSFw2 varied significantly between the studied groups(Plusions:Far distance clinical image quality parameters measured by PWS aberrometer differed significantly according to the technology of the implanted *** lISA Tri,SA60AT and PanOptix showed the highest values of far distance retinal image quality,while the lowest PSFw2 Strehl ratios were displayed by Miniwell,Mplus MF30 and Precizon Presbyopic.

关键词： Cataract surgery Pyramid.l aberrometry Multifocal IOl Extended depth of focus Accommodative IOl

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