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Usher syndrome:Genetic diagnosis and current therapeutic approaches

World journal of Otorhinolaryngology 2024年第1期11卷 1-17页

作者： Beatriz Rocha Cuzzuol jonathan Santos Apolonio Ronaldo Teixeira da Silva júnior Lorena Sousa de carvalho Luana Kauany de SáSantos Luciano Hasimoto Malheiro Marcel Silva Luz Mariana Santos Calmon Henrique de Lima Crivellaro Fabian Fellipe Bueno Lemos Fabrício Freire de Melo Instituto Multidisciplinar em Saúde Universidade Federal da BahiaVitória da Conquista 45029-094BahiaBrazil

Usher Syndrome(USH)is the most common deaf-blind syndrome,affecting approximately 1 in 6000 people in the deaf *** genetic condition is characterized by a combination of hearing loss(HL),retinitis pigmentosa,and,in some cases,vestibular *** the subtypes of USH,USH type 1 is considered the most severe form,presenting profound bilateral congenital deafness,vestibular areflexia,and early onset *** type 2 is the most common form,exhibiting congenital moderate to severe HL for low frequencies and severe to profound HL for high ***,type 3 is the rarest,initially manifesting mild symptoms during childhood that become more prominent in the first decades of *** dual impact of USH on both visual and auditory senses significantly impairs patients'quality of life,restricting their daily activities and interactions with *** date,9 genes have been confirmed so far for USH:MYO7A,USH1C,CDH23,PCDH15,USH1G,USH2A,ADGRV1,WHRN and *** genes are inherited in an autosomal recessive manner and encode proteins expressed in the inner ear and retina,leading to functional *** non-genetic methods can assist in patient triage and disease extension evaluation,genetic and molecular tests play a pivotal role in providing genetic counseling,enabling appropriate gene therapy,and facilitating timely cochlear implantation(CI).The CRISPR/Cas9 system and viral-based gene replacement therapy have recently emerged as highly promising techniques for treating *** drug therapy,PTC-124 and Nb54 have been identified as promising drug interventions for genetic HL in ***,CI has proven to be critical in the restoration of *** review aims to summarize the genetic and molecular diagnosis of USH and highlight the importance of early diagnosis in Cuzzuol BR et *** and current treatments of USH WjO https://*** 2 january 19,2024 Volume 11 Issue 1 guiding appropriate treatment strategies and i

关键词： Usher syndromes Hearing loss Retinitis pigmentosa Diagnosis Genetic therapies Cochlear implantation Quality of life

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Microfibrillated Cellulose from Sugarcane Bagasse as a Biorefinery Product for Ethanol Production

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journal of Renewable Materials 2018年第2期6卷 195-202页

作者： Rafael Grande Eliane Trovatti Maria Tereza B.Pimenta Antonio j.F.carvalho University of São Paulo Av.João Dagnone 1100CEP 13563-120São CarlosSão PauloBrazil University of Araraquara(UNIARA) R.Carlos Gomes1217CEP 14801-320AraraquaraSão PauloBrazil Suzano Papel e Celulose SA Av.Lirio Correa146506460-040LimeiraSão PauloBrazil

Research involving the preparation of microfibrillated cellulose(MFC)from sugarcane bagasse is a relevant topic to the production of new nanomaterials and more accessible cellulose substrates for the production of second generation *** the transformation of cellulose into glucose,the precursor of second generation ethanol,this nanosized cellulosic substrate represents a more appropriate material for the chemical hydrolysis *** high aspect ratio of MFC improves hydrolysis,requiring mild conditions and decreasing the generation of ***,MFC was prepared from sugarcane bagasse by ultrasound *** material was oxidized with 2,2,6,6-tetramethylpiperidine-N-oxyl(TEMPO)to produce negatively charged high defibrillated *** MFC was characterized by Fourier transform infrared spectroscopy(FTIR),X-ray diffraction(XRD),scanning electron microscopy(SEM)and viscosity measurements.

关键词： Cellulose nanofibrils substrate second generation ethanol

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Comparative Transcriptomic Profiling of Vitis vinifera Under High Light Using a Custom-Made Array and the Affymetrix GeneChip

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Molecular Plant 2011年第6期4卷 1038-1051页

作者： Luisa C. carvalho Belmiro j. Vilela Phil M. Mullineaux Sara Amancio CBAA Instituto Superior de Agronomia Universidade Tecnica de Lisboa Tapada da Ajuda 1349-017 Lisboa Portugal DDSB John Innes Centre Colney Norwich NR4 7UH UK Department of Biological Sciences University of Essex Colchester CO4 3SQ UK

Understanding abiotic stress responses is one of the most important issues in plant research nowadays. Abiotic stress, including excess light, can promote the onset of oxidative stress through the accumulation of reactive oxygen species. Oxidative stress also arises when in vitro propagated plants are exposed to high light upon transfer to ex vitro. To determine whether the underlying pathways activated at the transfer of in vitro grapevine to ex vitro conditions reflect the processes occurring upon light stress, we used Vitis vinifera Affymetrix GeneChip （VvGA） and a custom array of genes responsive to light stress （LSCA） detected by real-time reverse transcriptase PCR （qRT-PCR）. When gene-expression profiles were compared, ＇protein metabolism and modification＇, ＇signaling＇, and ＇anti-oxidative＂ genes were more represented in LSCA, while, in VvGA, ＇cell wall metabolism＇ and ＇secondary metabolism＇ were the categories in which gene expression varied more significantly. The above functional categories confirm previous studies involving other types of abiotic stresses, enhancing the common attributes of abiotic stress defense pathways. The LSCA analysis of our experimental system detected strong response of heat shock genes, particularly the protein rescuing mechanism involving the cooperation of two ATP-dependent chaperone systems, Hsp100 and Hsp70, which showed an unusually late response during the recovery period, of extreme relevance to remove non-functional, potentially harmful polypeptides arising from misfolding, denaturation, or aggregation brought about by stress. The success of LSCA also proves the feasibility of a custommade qRT-PCR approach, particularly for species for which no GeneChip is available and for researchers dealing with a specific and focused problem.

关键词： Quantitative real-time PCR Vitis vinifera GeneChip light stress Heat Shock Proteins oxidative and photooxidative stress.

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Merits and limitations of TiO2-based photocatalytic pretreatment of soils impacted by crude oil for expediting bioremediation

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Frontiers of Chemical Science and Engineering 2017年第3期11卷 387-394页

作者： Yu Yang Hassan javed Danning Zhang Deyi Li Roopa Kamath Kevin McVey Kanwartej Sra pedro j.j. Alvarez Department of Civil and Environmental Engineering Rice University Houston TX 77005 USA Department of Chemistry Rice University Houston TX 77005 USA Department of Civil and Environmental Engineering Tongji University Shanghai 200092 China Chevron Energy Technology Company Houston TX 77002 USA

Heavy hydrocarbons （HHCs） in soils impacted by crude oil spills are generally recalcitrant to biodegrada- tion due to their low bioavailability and complex chemical structure. In this study, soils were pretreated with varying concentrations of ultraviolet radiation A （UVA） or ultraviolet radiation C （UVC） activated titanium dioxide （TiO2）（1%-5%） under varying moisture conditions （0%- 300% water holding capacity （WHC）） to enhance biodegradation of HCCs and shorten remediation time- frames. We demonstrate that pretreatment of impacted soils with UVC-activated TiO2 in soil slurries could enhance bioremediation of HHCs. Total petroleum hydrocarbon （TPH） removal after 24 h exposure to UVC （254 nm and 4.8 mW/cm2） was （19.15：1.6）% in slurries with 300% WHC and 5 wt-% TiO2. TPH removal was non-selective in the C15-C36 range and increased with moisture content and TiO2 concentration. In a 10-d bioremediation test, TPH removal in treated soil increased to （26.05：0.9）%, compared to （15.45：0.8）% for controls without photo- catalytic pre-treatment. Enhanced biodegradation was also confirmed by respirometry. This suggests that addition of UVC-activated TiO2 to soil slurries can transform recalcitrant hydrocarbons into more bioavailable and biodegradable byproducts and increase the rate of subsequent biodegradation. However, similar results were not observed for soils pretreated with UVA activated TiO2. This suggests that activation of TiO2 by sunlight and direct addition of TiO2 to unsaturated soils within landfarming setting may not be a feasible approach. Nevertheless, less than 1% of UVA （7.5 mW/cm2） or UVC （1.4 mW/cm2） penetrated beyond 0.3 cm soil depth, indicating that limited light penetration through soil would hinder the ability of TiO2 to enhance soil bioremediation under land farming conditions.

关键词： ZiO2 pretreatment bioremediation total petroleum hydrocarbons ultraviolet

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Correction to:Effects of chemical sterilization of the culture media,porous membranes and luminosity on in vitro culture of Eucalyptus grandis×Eucalyptus urophylla

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journal of Forestry Research 2022年第5期33卷 1705-1707页

作者： Letícia Vaz Molinari Denys Matheus Santana Costa Souza Maria Lopes Martins Avelar Sérgio Bruno Fernandes Douglas Santos Gonçalves júlio Cézar Tannure Faria Dulcineia de carvalho Gilvano Ebling Brondani Department of Forestry Sciences Federal University of LavrasLavrasMinas GeraisBrazil

Correction to:***://***/10.1007/s11676-020-01240-5 In the original publication of the article,there were errors in the fig 3 and fig *** corrected Figs.3 and 6 are given below:The original article has been corrected.

关键词： corrected porous media

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Thermal Treatment of Hydrocarbon-Impacted Soils＂ A Review of Technology Innovation for Sustainable Remediation

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Engineering 2016年第4期2卷 426-437页

作者： julia E. Vidonish Kyriacos Zygourakis Caroline A. Masiello Gabriel Sabadell pedro j. j. Alvarez Department of Civil and Environmental Engineering Rice University Houston IX 77005 USA Department of Chemical and Biomolecular Engineering Rice University Houston TX 77005 USA Department of Earth Science Rice University Houston IX 77005 USA Chevron Energy Technology Company Houston IX 77002 USA

在受烃污染的土壤和底泥的修复处理工艺中,热处理工艺因其能快速且稳定地达到修复标准而占有重要地位。然而,持续高温属于能源密集型操作,且可破坏土壤性质,因此尽管此工艺被广泛应用,关于这些工艺的环境兼容性和可持续性却极少被报道... 详细信息

在受烃污染的土壤和底泥的修复处理工艺中,热处理工艺因其能快速且稳定地达到修复标准而占有重要地位。然而,持续高温属于能源密集型操作,且可破坏土壤性质,因此尽管此工艺被广泛应用,关于这些工艺的环境兼容性和可持续性却极少被报道。鉴于此,我们对几个针对烃污染土壤的常用热处理工艺进行了概述,对它们的潜在环境影响进行了评估;并基于能源需求、水需求、系统生态和土壤科学的全面考虑,提出了可持续且影响较小的发展框架。至今还没有一种通用适合的热处理工艺。工艺的恰当选择要根据污染状况(包括环境中存在的烃种类)和场地特征(如被污染土壤的性质、水含量和热敏性)而定。总之,处理工程与土壤科学、生态系统和植物生物学研究的有机结合是攻克技术瓶颈、提高去除率和促进热处理工艺可持续性发展的关键所在。

关键词：热处理技术土壤性质技术创新修复生态系统生态学受污染土壤碳氢化合物述评

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Lymphocyte subsets in alcoholic liver disease

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World journal of Hepatology 2013年第2期5卷 46-55页

作者： Luís Costa Matos Paulo Batista Nuno Monteiro joo Ribeiro Maria A Cipriano pedro Henriques Fernando Giro Armando carvalho Faculty of Medicine of the University of Coimbra 3004-504 CoimbraPortugal Centro Hospitalar Tondela Viseu E.P.E3504-509 ViseuPortugal Department of Anatomopathology University of Coimbra Hospital3004-504 CoimbraPortugal

AIM:To compare lymphocyte subsets between healthy controls and alcoholics with liver ***:The patient cohort for this study included individuals who were suspected to have alcoholic liver disease(ALD) and who had undergone liver biopsy(for disease grading and staging,doubts about diagnosis,or concurrent liver disease;n = 56).Normal controls included patients who were admitted for elective cholecystectomy due to non-complicated gallstones(n = 27).Formalin-fixed,paraffin-embedded liver biopsy specimens were sectioned and stained with hematoxylin and eosin and Perls' Prussian *** non-alcoholic steatohepatitis score was used to assess markers of *** population subsets were determined by flow cytometry.T lymphocytes were identified(CD3+),and then further subdivided into CD4+ or CD8+ populations.B lymphocytes(CD19+) and natural killer(NK) cell numbers were also *** addition to assessing lymphocyte subpopulation differences between ALD patients and controls,we also compared subsets of alcoholic patients without cirrhosis or abstinent cirrhotic patients to normal ***:The patient cohort primarily consisted of older *** alcoholism was present in 66.1%.Reported average daily alcohol intake was 164.9 g and the average lifetime cumulative intake was 2211.6 *** was present in 39.3% of the patients and 66.1% had significant fibrosis(perisinusoidal and portal/periportal fibrosis,bridging fibrosis,or cirrhosis) in their liver *** average Mayo end-stage liver disease score was *** hereditary hemochromatosis genotypes were *** patients(n = 56) presented with significant lymphopenia(1.5 × 109/L ± 0.5 × 109/L vs 2.1 × 109/L ± 0.5 × 109/L,P < 0.0001),due to a decrease in all lymphocyte subpopulations,except for NK lymphocytes:CD3+(1013.0 ± 406.2/mm3 vs 1523.0 ± 364.6/mm3,P < 0.0001),CD4+(713.5 ± 284.7/mm3 vs 992.4 ± 274.7/mm3,P < 0.0001),CD8+(262.3 ± 140.4/mm3 vs 478.9 ± 164.6/mm3,P < 0.0001),and CD19+(120.6

关键词： Lymphocyte subsets Flow cytometry Alcoholism Alcoholic liver disease Liver fibrosis Liver biopsy HFE gene

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A carbon- and oxygen-isotope record of recent environ- mental change from Qinghai Lake, NE Tibetan Plateau

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Chinese Science Bulletin 2003年第14期48卷 1463-1468页

作者： A.C.G.Henderson j.A.Holmes ZHANGjiawu M.j.Leng L.R.carvalho EnvironmentalChangeResearchCentre(ECRC) DepartmentofGeographyUniversityCollegeLondon26BedfordWayLondonWC1H0APUK CenterforAridEnvironmentandPaleoclimateResearch NationalLaboratoryofWesternChina'sEnvironmentalSystemsLanzhouUniversityLanzhou730000China NERCIsotopeGeosciencesLaboratory(NIGL) BritishGeologicalSurveyKeyworthNottingham.NG125GGUK Presentaddress:CentreforEcologyandHydrology(CEH) EdinburghBushEstatePenicuikMidlothianEH260OB.UK

A c. 300-year oxygen and carbon isotope record derived from fine-grained and ostracod carbonate from Qinghai Lake testifies to dramatic interannual tointerdecadal limnological change. Fine-grained carbonates, which are mainly authigenic, are likely to have formed in the epilimnion of the lake and their isotopic composition reflects the summer temperature and, more importantly, the isotopic composition of the near-surface waters, which is mainly a function of evaporative concentration. Ostracod shells are secreted in the benthos of the lake, and their isotopic composition reflects summer bottom-water conditions, together with fractionation effects, which may differ between species. Differences betweencontemporaneous values from authigenic carbonates and ostracod shells may provide an indication of stratification within the lake and variations in effective precipitation over the northeast part of the Tibetan Plateau over the past 300 years. A period of moderate evaporative concentration, from about 300 to 100 yr BP, was interrupted by a marked wet phase from ～100 to 40 yr BP, which was in turnfollowed by a return to drier conditions in the most recent part of the *** increase in ? 18O values in the latter part of the record accords well withinstrumental records of lake-level lowering and salinity increase since about 1955 AD.

关键词：中国西藏东北部环境变化碳同位素氧同位素季风古气候青海湖

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A call for international research on COVID-19-induced brain dysfunctions

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National Science Review 2021年第12期8卷 13-14页

作者： pedro A.Valdes-Sosa Alan C.Evans Mitchell j.Valdes-Sosa Mu-ming Poo Global Brain Consortium University of Electronic Sciences and Technology of China (UESTC) Cuban Neuroscience Center (CNEURO) Cuban Academy of Sciences Global Brain Consortium Royal Society of CanadaMc Gill Center for Integrative Neuroscience Mc Gill University Center for Excellence in Brain Science and Intelligence Technology Institute of NeuroscienceChinese Academy of Sciences

The COVID-19 pandemic, still raging worldwide, imposes staggering human and economic costs. These will only increase as many convalescent patients suffer consequences for many *** the virus can target multiple organ s...

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The aryl hydrocarbon receptor and the gut–brain axis

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Cellular & Molecular Immunology 2021年第2期18卷 259-268页

作者： Andreia Barroso joão Vitor Mahler pedro Henrique Fonseca-Castro Francisco j.Quintana Ann Romney Center for Neurologic Diseases Brigham and Women’s HospitalHarvard Medical SchoolBostonMAUSA Faculdade de Medicina FMUSP Universidade de São PauloSão PauloSPBrazil Broad Institute of MIT and Harvard CambridgeMAUSA

The aryl hydrocarbon receptor(AHR)is a ligand-activated transcription factor initially identified as the receptor for *** half a century after its discovery,AHR is now recognized as a receptor for multiple physiological ligands,with important roles in health and *** this review,we discuss the role of AHR in the gut–brain axis and its potential value as a therapeutic target for immune-mediated diseases.

关键词： Aryl hydrocarbon receptor Autoimmune diseases Infectious diseases cancer

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