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neurologic orphan diseases:Emerging innovations and role for genetic treatments

World Journal of Experimental Medicine 2023年第4期13卷 59-74页

作者： Ivelina P Kioutchoukova Devon T Foster Rajvi n Thakkar Marco A Foreman Brandon J Burgess rebecca M Toms Eduardo E Molina Valero Brandon Lucke-Wold College of Medicine University of FloridaGainesvilleFL 32611United States Florida International University Herbert Wertheim College of Medicine Florida International University Herbert Wertheim College of MedicineMiamiFL 33199United States Department of neurosurgery University of FloridaGainesvilleFL 32611United States

Orphan diseases are rare diseases that affect less than 200000 individuals within the United *** orphan diseases are of neurologic and genetic *** the current advances in technology,more funding has been devoted to developing therapeutic agents for patients with these *** our review,we highlight emerging options for patients with neurologic orphan diseases,specifically including diseases resulting in muscular deterioration,epilepsy,seizures,neurodegenerative movement disorders,inhibited cognitive development,neuron deterioration,and *** extensive literature review,gene therapy offers a promising route for the treatment of neurologic orphan *** use of clustered regularly interspaced palindromic repeats/Cas9 has demonstrated positive results in experiments investigating its role in several ***,the use of adeno-associated viral vectors has shown improvement in survival,motor function,and developmental milestones,while also demonstrating reversal of sensory ataxia and cardiomyopathy in Friedreich ataxia *** oligonucleotides have also been used in some neurologic orphan diseases with positive *** target of rapamycin inhibitors are currently being investigated and have reduced abnormal cell growth,proliferation,and *** innovations and the role of genetic treatments open a new window of opportunity for the treatment of neurologic orphan diseases.

关键词： neurologic orphan diseases Gene therapy Clustered regularly interspaced palindromic repeats/Cas9 Antisense oligonucleotides Adeno-associated virus mTOR inhibitors

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Transcriptomic and metabolomic analyses of cucumber fruit peels reveal a developmental increase in terpenoid glycosides associated with age-related resistance to Phytophthora capsici

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Horticulture Research 2017年第1期4卷 266-276页

作者： Ben n Mansfeld Marivi Colle Yunyan Kang A Daniel Jones rebecca Grumet Department of Horticulture Graduate Program in Plant BreedingGenetics and BiotechnologyMichigan State UniversityEast Lansing48824MIUSA College of Horticulture South China Agricultural UniversityGuangzhou510642China Department of Biochemistry and Molecular Biology Michigan State UniversityEast Lansing48824MIUSA Department of Chemistry Michigan State UniversityEast Lansing48824MIUSA

The oomycete,Phytophthora capsici,infects cucumber(Cucumis sativus L.)*** age-related resistance(ARR)to this pathogen was previously observed in fruit of cultivar‘Vlaspik’and shown to be associated with the *** fruits are highly susceptible,but develop resistance at~10–12 days post pollination(dpp).Peels from resistant(16 dpp)versus susceptible(8 dpp)age fruit are enriched with genes associated with defense,and methanolic extracts from resistant age peels inhibit pathogen *** we compared developing fruits from‘Vlaspik’with those of‘Gy14’,a line that does not exhibit *** analysis of peels of the two lines at 8 and 16 dpp identified 80 genes that were developmentally upregulated in resistant‘Vlaspik’16 dpp versus 8 dpp,but not in susceptible‘Gy14’at 16 dpp.A large number of these genes are annotated to be associated with defense and/or specialized metabolism,including four putative resistance(R)genes,and numerous genes involved in flavonoid and terpenoid synthesis and *** metabolomic analysis was performed on extracts from 8 and 16 dpp‘Vlaspik’and‘Gy14’fruit peels using Ultra-Performance Liquid Chromatography and Quadrupole Time-of-Flight Mass *** analysis of the metabolomes identified 113 ions uniquely abundant in resistant‘Vlaspik’16 dpp peel *** most abundant compounds in this group had relative mass defects consistent with terpenoid *** of the three most abundant ions were annotated as glycosylated nor-terpenoid ***,these analyses reveal potential mechanisms by which ARR to *** may be conferred.

关键词： metabolism resistance abundant

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Identifying RnA Modifications by Direct RnA Sequencing Reveals Complexity of Epitranscriptomic Dynamics in Rice

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Genomics, Proteomics & Bioinformatics 2023年第4期21卷 788-804页

作者： Feng Yu Huanhuan Qi Li Gao Sen Luo rebecca njeri Damaris Yinggen Ke Wenhua Wu Pingfang Yang State Key Laboratory of Biocatalysis and Enzyme Engineering School of Life SciencesHubei UniversityWuhan 430062China

Transcriptome analysis based on high-throughput sequencing of a cDnA library has been widely applied to functional genomic ***,the cDnA dependence of most RnA sequencing techniques constrains their ability to detect base modifications on RnA,which is an important element for the post-transcriptional regulation of gene *** comprehensively profile the n^(6)-methyladenosine(m^(6)A)and n^(5)-methylcytosine(m5 C)modifications on RnA,direct RnA sequencing(DRS)using the latest Oxford nanopore Technology was applied to analyze the transcriptome of six tissues in *** 94 million reads were generated,with an average length ranging from 619 nt to 1013 nt,and a total of 45,707 transcripts across 34,763 genes were *** profiles of transcripts at the isoform level were quantified among ***-wide mapping of m^(6)A and m5 C demonstrated that both modifications exhibited tissue-specific *** transcripts with m^(6)A modifications tended to be modified by m5 C,and the transcripts with modifications presented higher expression levels along with shorter poly(A)tails than transcripts without modifications,suggesting the complexity of gene expression *** Ontology analysis demonstrated that m^(6)A-and m5 C-modified transcripts were involved in central metabolic pathways related to the life cycle,with modifications on the target genes selected in a tissue-specific ***,most modified sites were located within quantitative trait loci that control important agronomic traits,highlighting the value of cloning functional *** results provide new insights into the expression regulation complexity and data resource of the transcriptome and epitranscriptome,improving our understanding of the rice genome.

关键词： Direct RnA sequencing Polyadenylated transcriptome n6-methyladenosine n5-methylcytosine Rice

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Phylogenetic definitions for 25 higher-level clade names of birds

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Avian Research 2022年第2期13卷 109-126页

作者： George Sangster Edward L.Braun Ulf S.Johansson rebecca T.Kimball Gerald Mayr Alexander Suh naturalis Biodiversity Center Darwinweg 2PO Box 95172300RALeidenthe Netherlands Department of Biology University of FloridaGainesvilleFL32607USA Department of Zoology Swedish Museum of Natural HistoryP.O.Box 50007SE–10405StockholmSweden Senckenberg Research Institute and natural History Museum Frankfurt Ornithological SectionSenckenberganlage 2560325Frankfurt am MainGermany School of Biological Sciences University of East AngliaNorwichNR47TUUK

Knowledge of the higher-level phylogenetic relationships of birds has grown substantially during the past two decades due to the application of genomic ***,the nomenclature of higher-level taxa has not become more stable,due to the lack of regulation of taxon names above the level of superfamily by the ICZn,and the usage of rank-based nomenclature,which is not tied to clades in a *** of regulation and the instability of rank-based nomenclature impede effective communication among *** review support for higher-level avian clades using a set of 10 phylogenomic data sets,and identify clades that are supported by congruency of at least four of *** provide formal definitions of the names of these clades based on the rules of the recently published *** names of 25 clades are here defined using minimum-crown-clade(n=23),minimum-clade(n=1)and maximum-crown-clade(n=1)*** new names are introduced here:Dinocrypturi,Pteroclimesites,Musophagotides,Phaethoquornithes and *** also review diagnostic apomorphies of the relevant clades,and identify known synonyms and *** establishing a formal link between higher-level taxon names and well-supported phylogenetic hypotheses,our phylogenetic definitions will provide a solid basis for the stabilization of avian higher-level nomenclature.

关键词： Aves Congruence PhyloCode Phylogenetic nomenclature Phylogenomics Rankless taxonomy

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Th1 cytokines promote T-cell binding to antigen-presenting cells via enhanced hyaluronan production and accumulation at the immune synapse

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Cellular & Molecular Immunology 2010年第3期7卷 211-220页

作者： Paul L Bollyky Stephen P Evanko rebecca P Wu Susan Potter-Perigo S Alice Long Brian Kinsella Helena Reijonen Kelly Guebtner Brandon Teng Christina K Chan Kathy R Braun John A Gebe Gerald T nepom Thomas n Wight Benaroya Research Institute SeattleWAUSA

Hyaluronan(HA)production by dendritic cells(DCs)is known to promote antigen presentation and to augment T-cell activation and *** hypothesized that pericellular HA can function as intercellular‘glue’directly mediating T cell–DC *** primary human cells,we observed HA-dependent binding between T cells and DCs,which was abrogated upon pre-treatment of the DCs with 4-methylumbelliferone(4-MU),an agent which blocks HA ***,T cells regulate HA production by DCs via T cell-derived cytokines in a T helper(Th)subset-specific manner,as demonstrated by the observation that cell-culture supernatants from Th1 but not Th2 clones promote HA *** effects were seen upon the addition of exogenous Th1 cytokines,IL-2,interferon c(IFn-c)and tumor necrosis factor a(TnF-a).The critical factors which determined the extent of DC–T cell binding in this system were the nature of the pre-treatment the DCs received and their capacity to synthesize HA,as T-cell clones which were pre-treated with monensin,added to block cytokine secretion,bound equivalently irrespective of their Th *** data support the existence of a feedforward loop wherein T-cell cytokines influence DC production of HA,which in turn affects the extent of DC–T cell *** also document the presence of focal deposits of HA at the immune synapse between T-cells and APC and on dendritic processes thought to be important in antigen *** data point to a pivotal role for HA in DC–T cell interactions at the IS.

关键词： dendritic cell hyaluronan immune synapse pericellular matrix Th1

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Hepatic sarcoidosis complicating treatment-naive viral hepatitis

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World Journal of Hepatology 2012年第12期4卷 402-405页

作者： Aloysious Aravinthan William Gelso Anita Limbu rebecca Brais Paul Richardson Department of Hepatology Cambridge University Hospitals NHS TrustCambridge CB2 0QQUnited Kingdom Department of Hepatology Cambridge University Hospitals NHS TrustCambridge CB2 0QQUnited Kingdom n Department of Histopathology Cambridge University Hospitals NHS TrustCambridge CB2 0QQUnited Kingdom Department of Hepatology The Royal Liverpool and Broadgreen University Hospitals NHS TrustLiverpool L7 8XPUnited Kingdom

Hepatic sarcoidosis is usually asymptomatic but rarely leads to adverse liver-related ***-existence of viral hepatitis and hepatic sarcoidosis is a rare,but recognised *** a balance between immune suppression and anti-viral therapy may be *** in the presence of viral hepatitis can lead to rapid deterioration of liver ***,anti-viral therapy may exacerbate granulomatous *** we present two cases of viral hepatitis co-existing with sarcoidosis that illustrate successful management *** one,hepatitis B replication was suppressed with oral antiviral therapy before commencing *** the second,remission of hepatic sarcoidosis was achieved with prednisolone,before treating hepatitis C and obtaining a sustained virological response with pegylated interferon and ribavirin therapy.

关键词： Hepatic sarcoidosis Chronic hepatitis C infection Chronic hepatitis B infection Immune suppression Anti-viral therapy

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Sequence analysis of peptides with biological activities using electrospray-Fourier transform ion cyclotron resonance mass spectrometry

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Chinese Science Bulletin 2001年第15期46卷 1281-1285页

作者： HE Meiyu XU Jiaxi HE Xiaoran Tak Wah Dominic Chan rebecca L.C.Lau Department of Chemistry Peking UniversityBeijing 100871China Beijing Mass Spectrometry Center Beijing 100080China Department of Chemistry The Chinese University of Hong KongShatinN.T.Hong KongChina

The mass spectra of five peptides with biological activities are reported. All mass spectra were recorded using a 4.7-T Fourier transform ion cyclotron resonance mass spectrometer equipped with an external electrospray source. The accurate molecular weights for the five peptides prepared by solid phase synthesis were measured as 1765.9013, 1063.5420, 1092.5254, 820.3804 and 1078.5193, respectively. All the data were obtained with the external calibration. Differences between observed and theoretical monoisotopic molecular weights were in the (0.2-1.0)×10-6 range. The complete primary sequence for the five polypep-tides were determined using the method of in-source electrospray ionization/collision induced dissociation (ESI/CID). All the intact y series ions and b series ions were obtained from various peptides respectively, thus determining the sequences of the five polypeptides. We found that the meas-ured accurate molecular mass of sample 4 was not in agreement with that expected from the planned

关键词： sequence peptide with biological activity Fourier transform ion cyclotron resonance mass spectrometry electrospray collision-induced dissociation.

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Metabolite profile comparisons between ascending and descending colon tissue in healthy adults

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World Journal of Gastroenterology 2020年第3期26卷 335-352页

作者： Bridget A Baxter Kristopher D Parker Michael J nosler Sangeeta Rao rebecca Craig Catherine Seiler Elizabeth P Ryan Department of Environmental and Radiological Health Sciences Colorado State UniversityFort CollinsFort CollinsCO 80523United States University of Colorado Health Gastroenterology Clinic Fort CollinsCO 80524United States Department of Clinical Sciences Colorado State UniversityFort CollinsCO 80523United States Harmony Surgery Center Fort CollinsCO 80528United States Director of Clinical Operations Harmony Surgery CenterFort CollinsCO 80523United States

BACKGROUnD Obesity is a risk factor for colorectal cancer,yet metabolic distinctions between healthy right and left colon tissue,before cancer is diagnosed,remains largely *** study compared right-ascending and left-descending colon tissue metabolomes to identify differences from the stool metabolome in normal weight,overweight,and obese *** To examine right and left colon tissue metabolites according to body mass index that may serve as mechanistic targets for interventions and biomarkers for colon cancer *** Global,non-targeted metabolomics was applied to assess right-ascending and left-descending colon tissue collected from healthy adults undergoing screening colonoscopies to test the hypothesis that BMI differentially impacts colon tissue metabolite *** colon tissue and stool metabolome of healthy adults(n=24)was analyzed for metabolite signatures and metabolic pathway networks implicated in progression of colorectal *** Ascending and descending colon contained 504 host,food,and microbiotaderived metabolites from normal weight,overweight and obese adults grouped according to body mass *** acids,lipids,and nucleotides were among the chemical types that further differentiated from the stool metabolite *** weight adults had 46 significantly different metabolites between ascending and descending colon tissue locations,whereas there were 37 metabolite differences in overweight and 28 metabolite differences for obese adults(Pne n-oxide,endocannabinoids and monoacylglycerols with different relative abundances identified between ascending and descending *** and secondary bile acids,vitamins,and fatty acids also showed marked relative abundance differences in colon tissue from overweight/obese *** There were metabolite profile differences between right-ascending and leftdescending colon tissue in healthy *** lipids and other metabolites in

关键词： Colon Ascending Descending Metabolomics Obesity Stool

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感官评价实践

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作者： (美)Herbert Stone (美)rebecca n. Bleibaum (美)Heather A. Thomas

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Measurement of(n,α)and(n,2n)reaction cross sections at a neutron energy of 14.92±0.02 MeV for potassium and copper with uncertainty propagation

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Chinese Physics C 2022年第1期46卷 110-117页

作者： A.Gandhi Aman Sharma rebecca Pachuau1 namrata Singh L.S.Danu S.V.Suryanarayana B.K.nayak A.Kumar Department of Physics Banaras Hindu UniversityVaranasi 221005India nuclear Physics Division Bhabha Atomic Research CentreMumbai 400085India

Experimentally measured neutron activation cross sections are presented for the ^(65)Cu(n,0)^(62m)Cu,^(41) K(n,a)^(38C)l,and ^(65)Cu(n.2n)^(64)Cu reactions with detailed uncertainty *** neutron cross secions were measured at an incident energy of 14.92±0.02 MeV,and the neutrons were based on the(d,n)a fusion *** ^(27) Al(n,a)^(24)na reaction was used as a reference reaction for the normalization of the neutron *** pre-calib-rated lead-shielded HPGe detector was used to detect the residues'γ-ray *** data from the measured cross sections are compared to the previously measured cross sections from the EXFOR database,theoretically calculated cross sections using the TALYS and EMPIRE codes,and evaluated nuclear data.

关键词： nuclear reactions (n,a)and(n,2n)reactions t(c/,n)a neutron source uncertainty propagation covariance analysis TALYS-1.9 and EMPIRE-3.2 code

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