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Update of the spectrum of mucopolysaccharidoses type Ⅲ in Tunisia: identification of three novel mutations and in silico structural analysis of the missense mutations

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World Journal of Pediatrics 2017年第4期13卷 374-380页

作者： Souad Ouesleti maria francisca coutinho Isaura Ribeiro Abdehedi Miled Dalila Saidane Mosbahi Sandra Alves Biochemical Service CHU Farhat Hached4000SousseTunisia Research and Development Unit Department of Human GeneticsNational Institute of Health Dr.Ricardo JorgePortoPortugal Biochemical Genetics Unit Medical Genetics Center Dr.Jacinto de Magalh(a)esPorto Hospital CentrePortoPortugal Laboratory of Analysis Treatment and Valorization of Pollutants of the Environment and ProductsFaculty of PharmacyUniversity of Monastir5000Tunisia

Background:Mucopolysaccharidoses type Ⅲ (MPS Ⅲ) are a group of autosomai recessive lysosomal storage diseases,caused by mutations in genes that code for enzymes involved in the lysosomal degradation of heparan sulphate:heparan sulfate sulfamidase (SGSH),a-N-acetylglucosaminidase (NAGLU),heparan sulfate acetyl-CoA:a-glucosaminide N-acetyltransferase (HGSNAT),and N-acetylglucosamine-6-sulfatase (GNS).Methods:In this study,we have performed the molecular analysis of the SGSH,NAGLU and HGSNAT genes in 10 patients from 6 different MPS Ⅲ Tunisian ***:In the SGSH gene,two mutations were identified:one novel (p.D477N) and one already described (p.Q365X).In the NAGLU gene,two novel mutations were discovered (p.L550P and p.E153X).For the novel missense mutations found in these two genes we performed an in silico structural analysis and the results were consistent with the clinical course of the patients harboring those ***,in HGSNAT gene,we found the splicesite mutation c.234+1G>A that had already been reported as relatively frequent in MPS ⅢC patients from countries surrounding the basin of the Mediterranean *** presence in two Tunisian MPS ⅢC families points to the hypothesis of its peri Mediterranean *** the exception of the c.234+1G>A mutation,that was identified in two unrelated MPS ⅢC families,the other identified mutations were family-specific and were always found in homozygosity in the patients studied,thus reflecting the existence of consanguinity in MPS Ⅲ Tunisian ***:Three novel mutations are reported here,further contributing to the knowledge of the molecular basis of these *** results of this study will allow carrier detection in affected families and prenatal molecular diagnosis,leading to an improvement in genetic counseling.

关键词： molecular characterization mucopolysaccharidosis Ⅲ mutation

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Effect of asphalt thin beams mixed with three nominal maximum aggregate sizes in the bending beam rheometer on the prediction of thermal properties of bituminous material

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Frontiers of Structural and Civil Engineering 2017年第1期11卷 1-7页

作者： Chun-Hsing HO maria francisca Martinez GONZALEZ Cristina Pilar Martin LINARES Department of Civil Engineering Construction Management and Environmental Engineering Northern Arizona UniversityFlagstaff Arizona 86001 USA

The objective of this research is to evaluate an impact of asphalt mixture beams with varying sizes of aggregate in the Bending Beam Rheometer （BBR） for testing/predicting thermal cracking properties of asphalt pavements. The BBR test has following benefits： the equipment is cheaper, it uses smaller specimens, faster conditioning, easier availability for quality control, easier to manage, etc. However some concerns have been raised： some consider that the size of the aggregate may affect the test＇s results; the other concern is that such small beams cannot represent the whole properties of the asphalt pavement. To address these criticisms, imaging techniques, statistical analysis, and viscoelastic modeling are used. Asphalt thin beams prepared with three different nominal maximum aggregate size （NMAS）（12.5mm, 9.5 mm, and 4.75 mm） were tested at three different temperatures （-18℃, -24℃, and -30℃）. Based on results from statistical analyses and viscoelasticity, the ratio of asphalt binders and voids and stiffness differences among the three NMAS specimens are not significant, meaning that the impact of asphalt thin beams prepared with the three NMAS on the prediction of thermal cracking is minimal and can be neglected.

关键词： bending beam rheometer thin beams thermal cracking

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Bioactive polysaccharides from medicinal mushrooms:A review on their isolation,structural characteristics and antitumor activity

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Food Bioscience 2022年第5期49卷 119-129页

作者： Juliana Garcia francisca Rodrigues maria Jos´e Saavedra Fernando M.Nunes Guilhermina Marques CITAB-Centre for the Research and Technology of Agro-Environment and Biological Sciences University of Tr´as-os-Montes e Alto Douro5001-801Vila RealPortugal AquaValor-Centro de Valorizaç˜ao e Transferˆencia de Tecnologia da´Agua-Associaç˜ao Rua Dr.Júlio Martins n.◦15400-342ChavesPortugal REQUIMTE/LAQV-Polytechnic of Porto-School of Engineering Rua Dr.Ant´onio Bernardino de Almeida4314249-015PortoPortugal CQ-VR-Centro de Química-Vila Real Food and Wine Chemistry Lab.Chemistry DepartamentUniversity of Tr´as-os-Montes e Alto Douro5001-801Vila RealPortugal

Mushrooms either alone or in combination with conventional cancer treatment have been enhanced the efficacy of chemotherapy and radiotherapy improving the patients’quality of *** therapy effects have been attributed to polysaccharides;the most potent compounds found in the fungi *** to their wide range of biological activity and the backbone of glucose residues linked byβ-(1→3)-glycosidic bonds,often with attached side-chain glucose residues joined byβ-(1→6)linkages,the mushroom’s polysaccharides,particularly theβ-glucans,are the most versatile ***,D-fraction,and schizophyllan from Lentinula edodes,Grifola frondosa,and Schizophyllum commune,respectively are well-knownβ-glucans,however,the precise immunomodulation and anticancer mechanisms of action remain to be *** to several studies,β-glucan can interact with certain receptors on macrophages and dendritic cells,such as dectin-1 and TLRs,producing different cytokines and,in turn,indirectly activating other immune cells including T and B ***,recent findings have proved thatβ-glucans have a direct cytotoxic effect on cancer cells suppressing proliferation and enhancing apoptosis possibly via multiple pathways such as PI3K/Akt/mTOR,NF-κB-,ERK-,ERα-,caspase-and p53-dependent ***,this study intends to provide information on the lentinan,D-fraction,and schizophyllan by examining the extraction procedures,chemical composition,and immunostimulatory and antitumoral biological *** research directions should be directed toward improving the validity and reliability of randomized trials to confirm the potential role ofβ-glucans on the immune system and as anticancer therapy.

关键词： Lentinan D-fraction Schizophyllan Anticancer activity

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PillCam COLON 2~ in Crohn's disease:A new concept of pan-enteric mucosal healing assessment

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World Journal of Gastroenterology 2015年第23期21卷 7233-7241页

作者： Pedro Boal Carvalho Bruno Rosa francisca Dias de Castro maria Joo Moreira José Cotter Life and Health Sciences Research Institute School of Health Sciences University of Minho ICVS/3B’s PT Government Associate Laboratory Gastroenterology Department Centro Hospitalar do Alto Ave

AIM: To evaluate mucosal healing in patients with small bowel plus colonic Crohn's disease(CD) with a single non-invasive examination, by using PillCam COLON 2.(PCC2).METHODS: Patients with non-stricturing nonpenetrating small bowel plus colonic CD in sustained corticosteroid-free remission were included. At diagnosis,patients had undergone ileocolonoscopy to identify active CD lesions, such as ulcers and erosions, and small bowel capsule endoscopy to assess the Lewis Score(LS). After ≥ 1 year of follow-up, patients underwent entire gastrointestinal tract evaluation with PCC2. The primary endpoint was assessment of CD mucosal healing, defined as no active colonic CD lesions and LS < ***: Twelve patients were included(7 male;mean age: 32 years), and mean follow-up was 38 *** majority of patients(83.3%) received immunosuppressive therapy. Three patients(25%) achieved mucosal healing in both the small bowel and the colon,while disease activity was limited to either the small bowel or the colon in 5 patients(42%). It was possible to observe the entire gastrointestinal tract in 10 of the12 patients(83%) who underwent ***: Only three patients in sustainedcorticosteroid-free clinical remission achieved mucosal healing in both the small bowel and the colon, highlighting the limitations of clinical assessment when stratifying disease activity, and the need for pan-enteric endoscopy to guide therapeutic modification.

关键词： Crohn's disease Mucosal healing Capsuleendoscopy Small bowel diseases Inflammatory boweldisease

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PillCam~ SB3 capsule: Does the increased frame rate eliminate the risk of missing lesions?

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World Journal of Gastroenterology 2016年第10期22卷 3066-3068页

作者： Sara Monteiro francisca Dias de Castro Pedro Boal Carvalho maria Joao Moreira Bruno Rosa José Cotter Department of Gastroenterology Hospital Senhora da Oliveira Rua dos Cutileiros Creixomil 4835-044 Guimaraes Portugal Life and Health Sciences Research Institute (ICVS) School of Health Sciences University of Minho 4710-057 Braga Portugal ICVS/3B's PT Government Associate Laboratory 4710-057 Braga/Guimaraes Portugal

Since its emergence in 2000, small bowel capsule endoscopy(SBCE) has assumed a pivotal role as an investigation method for small bowel diseases. The PillCam SB2-ex offers 12 h of battery time, 4 more than the previous version(SB2). Rahman et al recently found that the PillCam SB2-ex has a significantly increased completion rate, although without higher diagnostic yield, compared with the SB2. We would like to discuss these somewhat surprising results and the new potentialities of the PillCam SB3 regarding the diagnostic yield of small bowel studies. PillCam SB3 offers improved image resolution and faster adaptable frame rate over previous versions of SBCE. We recently compared the major duodenal papilla detection rate obtained with PillCam SB3 and SB2 as a surrogate indicator of diagnostic yield in the proximal small bowel. The PillCam SB3 had a significantly higher major duodenal papilla detection rate than the PillCam SB2(42.7% vs 24%, P = 0.015). Thus, the most recent version of the PillCam capsule, SB3, may increase diagnostic yield, particularly in the proximal segments of the small bowel.

关键词： PillCam~ SB2 PillCam~ SB3 Capsule endoscopy Diagnostic yield Lesions Frames

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Case report of a criminal dismemberment in Northeast Brazil

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Forensic Sciences Research 2022年第4期7卷 637-642页

作者： Evelyne Pessoa Soriano maria do Socorro Dantas de Araújo francisca de Assis Nascimento Pereira francisca Divina Silveira de Melo Cristiane Helena da Silva Barbosa Freire Marcus Vitor Diniz de Carvalho Medico-legal Institute of João Pessoa ParaíbaBrazil Faculty of Dentistry(FOP) Center for Studies in Forensic Anthropology(CEAF)University of Pernambuco(UPE)RecifeBrazil

Several decomposed body parts were received for examination by the Forensic Anthropology section of the Medico-legal Institute of João Pessoa,Paraíba,*** portions of the lower and upper limbs,ribs,vertebrae,and a skull were thoroughly *** biological profile indicated a male individual with an estimated age range between 23 and 57 years and a mean age of 35.2 years(SD=9.4;phase IV,Suchey-Brooks).The skeleton showed injuries caused by sharp force and sharp-blunt force trauma that affected all body ***,the lesions are mainly in the diaphyseal segments of the long bones,sacrum,pelvis,mandible,maxilla,scapulae,sternum,vertebrae,the distal epiphysis of the left fibula,and the distal epiphysis of the left tibia displayed characteristics compatible with injuries produced *** was not possible to determine the cause of *** resulted in a positive *** of common difficulties faced in forensic practice,it is often not possible for forensic anthropologists to go to the crime scene,X-ray or body scanners are frequently unavailable,and the victim’s medical and/or dental records are sometimes *** difficulties make identification ultimately depend on genetic analysis,which is more time-consuming than other identification *** this,bone fragment examination in dismemberment cases is a complex *** Anthropology can shed light on cases involving the identification of dismembered remains,which are challenging because of the number of traumatic injuries,as well as different injury patterns,on bones.

关键词： Forensic sciences forensic anthropology dismemberment human identification

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Lactic Acid Fermentation of Peppers

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Food and Nutrition Sciences 2013年第11期4卷 47-55页

作者： maria Rosa Alberto maria francisca Perera Mario Eduardo Arena Facultad de Bioquímica Química y Farmacia Universidad Nacional de Tucumán (UNT) Tucumán Argentina Facultad de Bioquímica Química y Farmacia Universidad Nacional de Tucumán (UNT) Tucumán Argentina Centro Científico Tecnológico Tucumán—Consejo Nacional de Investigaciones Científicas y Tecnológicas (CCT-CONICET) Tucumán Argentina. Facultad de Bioquímica Química y Farmacia Universidad Nacional de Tucumán (UNT) Tucumán Argentina Centro Científico Tecnológico Tucumán—Consejo Nacional de Investigaciones Científicas y Tecnológicas (CCT-CONICET) Tucumán Argentina.

Different peppers fermentations (Capsicum annum, grossum variety) were assayed: spontaneous, native microflora supplemented individually with Lactobacillus plantarum N8, Leuconostoc mesentereroides L. or Pediococcus pentosaceus 12p and by pure or combined cultures of these lactic acid bacteria (LAB). In order to eliminate the native flora, different kinds of heat treatment were assayed. The treatment selected was heating in autoclaved after research 3/4 atmosphere and to turn off. Fermentations were carried out at 22?C and 30?C and the culture media contained 2% or 0.2% glucose and 4% NaCl. Sugar consumption, pH reduction and acid production were higher at 30℃ than at 22℃. At both temperatures, spontaneous fermentation showed a slower rate reduction in pH than inoculated samples. Diminution in pH in presence of 2% glucose was faster than at 0.2%, but minimum pH was in both case lower than 3.0. Maximum growth was reached between 2 and 5 days of fermentation in all the samples assayed. After 30 days of incubation in presence of 2% glucose the survival of LAB was nearly 5 log ufc/ml. The survival was higher at the lower temperature assayed for both glucose concentrations. Organoleptic properties of peppers fermented with a mixed culture of Leuconostoc mesenteroides and Pediococcus pentosaceus were found best by a human panel. This sample has a relation lactic acid/acetic acid of nearly 3 in the conditions assayed.

关键词： Fermentation Lactic Acid Bacteria Peppers Capsicum annum

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Decreased prevalence of celiac disease among Brazilian elderly

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World Journal of Gastroenterology 2013年第12期19卷 1930-1935页

作者： Lucas Malta Almeida Luiz Claudio Castro Rosa Harumi Uenishi Fernanda coutinho de Almeida Patricia maria Fritsch Lenora Gandolfi Riccardo Pratesi Yanna Karla de Medeiros Nóbrega Graduate Program in Medical Sciences University of Brasilia School of Medicin Doctoral fellow of Coordenao de Aperfeioamento de Pessoal de Nível Superior Graduate Program in Health Sciences University of Brasilia School of Health Sciences Research Center for Celiac disease University of Brasilia School of Medicine Department of Pharmaceutical Sciences University of Brasilia School of Health Sciences Research Center for Celiac Disease University of Brasilia School of Medicine

AIM: To evaluate the prevalence of celiac disease in a group of Brazilian individuals over 60 years of age and compare it with the previously known prevalence in a pediatric group living in the same geographical ***: The research protocol was approved by the Ethics Committee of the University of Brasilia School of Medicine, Brasilia, Brazil. Blood samples from 946 individuals (295 male and 651 female) aged 60 years or older were collected between May 2010 and July 2011. The study subjects' mean and median ages were 68.1 and 67 years, respectively, ranging from 60 to 92 years. That age distribution closely corresponded to the age distribution of the Brazilian population according to the Brazilian 2010 census. The participants were consecutive and unselected outpatients undergoing blood tests at the University of Brasilia Hospital's Clinical Pathology Laboratory. All sera were tested for immunoglobulin A anti-transglutaminase antibodies (IgA-tTG) by enzymelinked immunosorbent assay, and those that were positive were further tested for immunoglobulin A antiendomysium antibodies (IgA-EMA). Human leukocyte antigen (HLA) genotyping was performed for all individuals who exhibited positive serologic results for IgA-tTG and/or ***: Out of the 946 studied patients, only one previously diagnosed case of biopsy-proven celiac disease was detected. For the remaining subjects, nine serum samples tested positive for IgA-tTG antibodies; however, none of them tested positive for IgA-EMA antibodies. The HLA genotyping of those nine subjects revealed that one was carrying DQA1*0501 and two were carrying DQB1*0201 alleles. These data showed that, among those 946 elderly individuals, the prevalence of celiac disease (CD) was 0.1% (95%CI: 0.00-0.59). The prevalence of CD for the elderly group was compared with that observed for the group of 2034 children younger than 15 years (age range, 1-14 years; mean age, 8 years) who took part in our previous CD prevalence screen

关键词： Celiac disease Gluten-sensitive enteropathy Epidemiology Elderly Mortality

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Polyphenolic composition, antibacterial, modulator and neuroprotective activity of Tarenaya spinosa(Jacq.) Raf.(Cleomaceae)

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Asian Pacific Journal of Tropical Biomedicine 2019年第1期9卷 12-17页

作者： francisca Samara Muniz dos Santos Jose Weverton Almeida Bezerra Jean Paul Kamdem Aline Augusti Boligon Marli Matiko Anraku Ana Raquel Pereira da Silva Kleber Ribeiro Fidelis Nadghia Figueiredo Leite Antonio Ivanildo Pinho Henrique Douglas Melo coutinho Jose Edilson Goncalves dos Santos Microscopy Laboratory Regional University of Cariri (URCA) Program Post graduate in Plant Biology (UFPE) Phytochemical Laboratory Department of Industrial Pharmacy Federal University of Santa Maria (UFSM) Microbiology and Molecular Biology Laboratory Regional University of Cariri (URCA)

Objective: To evaluate the antibacterial activity and neuroprotective capacity of the ethanolic and aqueous extracts of Tarenaya spinosa(T. spinosa) as well as to determine and quantify some of its polyphenols by high performance liquid chromatography with diode-array detection(HPLC-DAD). Methods: The bacterial Escherichia coli, Staphylococcus aureus and Pseudomonas aeruginosa strains, grown in Heart Agar Infusion, were tested. The drugs gentamicin, norfloxacin and imipenem were used to evaluate the modulating or antagonistic capacity of the T. spinosa extracts. The extract was analysed by HPLC-DAD to determine the main phenolic compounds. For the cell viability tests, individual heads of the Nauphoeta cinerea arthropod model were removed, homogenized in Trifluoromethyl ketone and centrifuged afterwards. Subsequently, 20 μL of NaN O2 were added to the biological material, except in the control group, to evaluate the protection capacity of the extracts. The homogenate of the insect heads was incubated for 2 h in tubes containing tetrazolium bromide. Results: HPLC-DAD demonstrated that the ethanolic extract of T. spinosa presented caffeic acid as the major compound. The ethanolic extract also showed neuroprotective effects at concentrations ≥ 10 μg/mL, while aqueous extract was shown to have a protective effect only at the concentration of 100 μg/mL. The aqueous extract demonstrated a clinically relevant antibacterial activity against the Staphylococcus aureus multidrug resistant strain-MDR, with MIC 512 μg/m L. However, when the extracts were associated with gentamicin and imipenem, a synergism was detected against Staphylococcus aureus and Escherichia coli MDR strains. Conclusions: Although it does not present an antibacterial action, the extracts of T. spinosa can be used in the pharmaceutical industries since its extracts show modulating action of drugs. Besides, these natural products have neuroprotective capacity.

关键词： Tarenaya spinosa Cleome spinose Nauphoeta cinerea Synergism Antibiotic resistance

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Systematically Monitoring,Relational Database and Technology Roadmapping for Trends and Innovation Opportunities in Biopolymers

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Journal of Renewable Materials 2019年第11期7卷 1221-1230页

作者： Selma B.Jaconis Augusto T.Morita Paulo L.A.coutinho Suzana Borschiver Federal University of Rio de Janeiro Avenida Athos da Silveira Ramos149Ilha do FundãoRJ21941-909Brazil SENAI Mario Amato Rua Vitória Maria Medice Ramos330SBCSP09861-640Brazil SENAI CETIQT Rua Magalhães Castro174RiachueloRJ20961-020Brazil

In recent years environmental and sustainability concerns have impacted the global chemical industry and instituted a rush to produce products from renewable raw *** dynamic,complex and turbulent organizational scenario,around themes touching on the issue of sustainable development model,was created involving a large number of different actors:chemical/petrochemical industries,agroindustry companies,oil/gas companies,brand owners and end users,biotechnology startups,governments,universities and *** paper proposed the application of a structured and dynamic method of technological prediction for biopolymers in three levels:systematic monitoring process,relational database and the“alive”Technology Roadmapping visualization *** main objective is to identify strategic actions,business models,the latest´s technologies in development,as well as trends in the field of biopolymers in order to support companies on position themselves in this competitive ***,companies,universities,government agencies and institutions could apply this dynamic and alive methodology to indeed access innovation opportunities,challenges and threats for different industrial segments and to provide dynamic knowledge management collaborating to their strategy including a database crossing for the all organization.

关键词： Biopolymers sustainability renewable systematically monitoring forecasting technology roadmapping knowledge management innovation

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