Search results for "PSI"

showing 10 items of 10516 documents

Hierarchical architecture of sponge spicules: biocatalytic and structure-directing activity of silicatein proteins as model for bioinspired applicati…

2016

Since the first description of the silicateins, a group of enzymes that mediate the formation of the amorphous, hydrated biosilica of the skeleton of the siliceous sponges, much progress has been achieved in the understanding of this biomineralization process. These discoveries include, beside the proof of the enzymatic nature of the sponge biosilica formation, the dual property of the enzyme, to act both as a structure-forming and structure-guiding protein, and the demonstration that the initial product of silicatein is a soft, gel-like material that has to undergo a maturation process during which it achieves its favorable physical-chemical properties allowing the development of various t…

0301 basic medicinebiologyProtein ConformationChemistryBiophysicsNanotechnologybiology.organism_classificationCathepsinsBiochemistryPorifera03 medical and health sciencesSponge030104 developmental biologySponge spiculeBiomimetic MaterialsAnimalsMolecular MedicineMaturation processEngineering (miscellaneous)BiotechnologyBiomineralizationBioinspiration & Biomimetics
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Alexander Disease Mutations Produce Cells with Coexpression of Glial Fibrillary Acidic Protein and NG2 in Neurosphere Cultures and Inhibit Differenti…

2017

Background Alexander disease (AxD) is a rare disease caused by mutations in the gene encoding glial fibrillary acidic protein (GFAP). The disease is characterized by presence of GFAP aggregates in the cytoplasm of astrocytes and loss of myelin. Objectives Determine the effect of AxD-related mutations on adult neurogenesis. Methods We transfected different types of mutant GFAP into neurospheres using the nucleofection technique. Results We find that mutations may cause coexpression of GFAP and NG2 in neurosphere cultures, which would inhibit the differentiation of precursors into oligodendrocytes and thus explain the myelin loss occurring in the disease. Transfection produces cells that diff…

0301 basic medicinecaspase-3Cathepsin Dmacromolecular substancesHSP27lcsh:RC346-429oligodendrocyte precursors03 medical and health sciencesMyelin0302 clinical medicineAlexander diseaseNG2Neurosphereneurospheresmedicinecathepsinlcsh:Neurology. Diseases of the nervous systemOriginal ResearchGlial fibrillary acidic proteinbiologyNeurogenesisNestinGFAP stainmedicine.diseaseMolecular biologyAlexander disease030104 developmental biologymedicine.anatomical_structurenervous systemNeurologyglial fibrillary acidic proteinbiology.proteinNeurology (clinical)030217 neurology & neurosurgeryNeuroscienceFrontiers in Neurology
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Arabidopsis Serine Decarboxylase 1 (SDC1) in Phospholipid and Amino Acid Metabolism

2018

Arabidopsis thaliana serine decarboxylase 1 (SDC1) catalyzes conversion of serine to ethanolamine, the first reaction step of phosphatidylcholine and phosphatidylethanolamine biosynthesis. However, an involvement of SDC1 in amino acid metabolism remains elusive despite that serine is the substrate of SDC1. Here, we showed that SDC1 localizes in mitochondria although phosphatidylcholine and phosphatidylethanolamine are known to be produced in the endoplasmic reticulum (ER). Moreover, we found that overexpression of SDC1 decreased levels of amino acid compounds derived from mitochondrial tricarboxylic acid cycle. These results suggest that mitochondria-localized SDC1 plays an important role i…

0301 basic medicinechemistry.chemical_classificationPhosphatidylethanolamineArabidopsis thalianaEndoplasmic reticulumPhospholipidPlant ScienceMetabolismlcsh:Plant cultureAmino acidSerineCitric acid cycle03 medical and health scienceschemistry.chemical_compound030104 developmental biologychemistryBiochemistryBiosynthesislcsh:SB1-1110phospholipid biosynthesisserine decarboxylaseglycerolipid metabolismphospholipidOriginal ResearchFrontiers in Plant Science
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An Ethanolic Extract of Ampelopsis Radix Exerts Anti-colorectal Cancer Effects and Potently Inhibits STAT3 Signaling In Vitro

2017

Colorectal cancer (CRC) is a leading cause of cancer-related morbidity and mortality worldwide. Signal transducer and activator of transcription 3 (STAT3) signaling is constantly activated in CRC, and has been proposed as a pathogenic factor and a therapeutic target of CRC. Ampelopsis Radix (AR), a traditional Chinese medicinal herb, possesses low toxicity and has long been used clinically for the treatment of cancers including CRC. Some constituents of AR have been reported to exert anticancer properties by targeting STAT3. However, the anti-CRC mode and mechanism of action of AR have not been fully elucidated. Here, we investigated the involvement of STAT3 signaling in the anti-CRC effect…

0301 basic medicinecolorectal cancerBiologyPharmacologymigrationSTAT303 medical and health sciences0302 clinical medicinemedicineCytotoxic T cellPharmacology (medical)STAT3neoplasmsOriginal ResearchPharmacologyCell growthlcsh:RM1-950apoptosisCell migrationAmpelopsis Radixinvasiondigestive system diseaseslcsh:Therapeutics. Pharmacology030104 developmental biologyMechanism of actionApoptosis030220 oncology & carcinogenesisSTAT proteinbiology.proteinPhosphorylationmedicine.symptomFrontiers in Pharmacology
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The quality of life in girls with Rett syndrome

2016

Nowadays, quality of life is receiving an increasing attention in all scientific areas. Rett syndrome (RTT) is a rare neurological development, affecting mainly females. The congenital disease affects the central nervous system, and is one of the most common causes of severe intellectual disability. The aim of our study is to evaluate the effect of RTT on the quality of life of people who are affected. Both parents of 18 subjects, all female, diagnosed with RTT, took part in the research. Quality of life was assessed using the Italian version of the Impact of Childhood Illness Scale. This scale consists of 30 questions that investigate the effect of illness on children, parents and families…

0301 basic medicinecongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtylcsh:RC435-571lcsh:MedicineRett syndromeArticle03 medical and health sciencesRett syndrome0302 clinical medicineQuality of lifelcsh:PsychiatryIntellectual disabilitymedicineMedical historyPsychiatrylcsh:Rmedicine.diseaseSettore MED/39 - Neuropsichiatria InfantilePsychiatry and Mental health030104 developmental biologyquality of lifeintellectual disabilityScale (social sciences)Congenital diseasePsychology030217 neurology & neurosurgeryMental Illness
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Evolutionary Analysis of DELLA-Associated Transcriptional Networks

2017

DELLA proteins are transcriptional regulators present in all land plants which have been shown to modulate the activity of over 100 transcription factors in Arabidopsis, involved in multiple physiological and developmental processes. It has been proposed that DELLAs transduce environmental information to pre-wired transcriptional circuits because their stability is regulated by gibberellins (GAs), whose homeostasis largely depends on environmental signals. The ability of GAs to promote DELLA degradation coincides with the origin of vascular plants, but the presence of DELLAs in other land plants poses at least two questions: what regulatory properties have DELLAs provided to the behavior of…

0301 basic medicineevo–devoChlamydomonas reinhardtiiPlant ScienceBiologylcsh:Plant culturePhyscomitrella patensGene co-expression networks03 medical and health sciencesTranscriptional regulationArabidopsisBotanyTranscriptional regulationBIOQUIMICA Y BIOLOGIA MOLECULARArabidopsis thalianalcsh:SB1-1110Transcription factorIntegrative molecular systems biologyOriginal ResearchEvo-devofood and beveragesPlant signalingbiology.organism_classificationCell biologyGENETICA030104 developmental biologyEvolutionary developmental biologyFunction (biology)
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Nitration of Wheat Amylase Trypsin Inhibitors Increases Their Innate and Adaptive Immunostimulatory Potential

2018

Amylase trypsin inhibitors (ATI) can be found in all gluten containing cereals and are, therefore, ingredient of basic foods like bread or pasta. In the gut ATI can mediate innate immunity via activation of the Toll-like receptor 4 (TLR4) on immune cells residing in the lamina propria, promoting intestinal, as well as extra-intestinal, inflammation. Inflammatory conditions can induce formation of peroxynitrite (ONOO-) and, thereby, endogenous protein nitration in the body. Moreover, air pollutants like ozone (O3) and nitrogen dioxide (NO2) can cause exogenous protein nitration in the environment. Both reaction pathways may lead to the nitration of ATI. To investigate if and how nitration mo…

0301 basic medicinelcsh:Immunologic diseases. AllergyCell SurvivalT cellnon-celiac wheat sensitivityImmunologyInflammationAdaptive ImmunityImmunophenotyping03 medical and health scienceschemistry.chemical_compound0302 clinical medicineImmune systemprotein nitrationT-Lymphocyte SubsetsNitrationCell Line TumorwheatmedicineImmunology and AllergyHumansamylase trypsin inhibitorsTriticumPlant ProteinsOriginal ResearchInnate immune systemMacrophagesfood and beveragesDendritic CellsTetranitromethaneallergyImmunity InnateToll-Like Receptor 4030104 developmental biologymedicine.anatomical_structurechemistryBiochemistryAmylasesTLR4Cytokinesmedicine.symptomlcsh:RC581-607Trypsin InhibitorsPeroxynitriteBiomarkers030215 immunologyFrontiers in immunology
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A novel GABRB3 variant in Dravet syndrome: Case report and literature review

2020

Abstract Background Mutations in GABRB3 have been identified in subjects with different types of epilepsy and epileptic syndromes, including West syndrome (WS), Dravet syndrome (DS), Lennox‐Gastaut syndrome (LGS), myoclonic‐atonic epilepsy (MAE), and others. Methods and results We herewith report on a girl affected by DS, who has been followed from infancy to the current age of 18 years. Next‐generation sequencing (NGS)‐based genetic testing for multigene analysis of neurodevelopmental disorders identified two likely de novo pathogenic mutations, a missense variant in GABRB3 gene (c.842 C>T; p.Thr281IIe) and a nonsense variant found in BBS4 gene (c.883 C>T; p.Arg295Ter). Conclusion A likely…

0301 basic medicinelcsh:QH426-470media_common.quotation_subjectNonsenseMutation MissenseEpilepsies Myoclonic030105 genetics & hereditymedicine.disease_causeClinical ReportsBBS4 gene03 medical and health sciencesEpilepsyDravet syndromeGeneticsMedicineMissense mutationHumansMolecular BiologyGeneGenetics (clinical)media_commonGenetic testingGeneticsMutationClinical Reportmedicine.diagnostic_testbusiness.industryGABRB3 GeneEpileptic EncephalopathiesWest Syndromemedicine.diseaseReceptors GABA-ADravet syndromelcsh:Genetics030104 developmental biologyPhenotypeCodon NonsenseChild PreschoolFemalebusinessMicrotubule-Associated Proteins
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MARTX Toxin in the Zoonotic Serovar of Vibrio vulnificus Triggers an Early Cytokine Storm in Mice

2017

Vibrio vulnificus biotype 2-serovar E is a zoonotic clonal complex that can cause death by sepsis in humans and fish. Unlike other biotypes, Bt2 produces a unique type of MARTXVv (Multifunctional-Autoprocessive-Repeats-in-Toxin; RtxA13), which is encoded by a gene duplicated in the pVvBt2 plasmid and chromosome II. In this work, we analyzed the activity of this toxin and its role in human sepsis by performing in vitro, ex vivo, and in vivo assays. First, we demonstrated that the ACD domain, present exclusively in this toxin variant, effectively has an actin-cross-linking activity. Second, we determined that the whole toxin caused death of human endotheliocytes and monocytes by lysis and apo…

0301 basic medicinelcsh:QR1-502MicrobiologiaVibrio vulnificusmedicine.disease_causeMonocyteslcsh:MicrobiologysepsisVibrio vulnificusCells CulturedOriginal ResearchMice Inbred BALB CCell DeathVirulencebiologyqPCR arrayInfectious DiseasesHost-Pathogen InteractionsBacteris patògensCytokinesV. vulnificusFemaleMicrobiology (medical)Virulence FactorsBacterial ToxinsImmunologyVirulenceSerogroupMicrobiologyMicrobiology03 medical and health sciencesImmune systemMARTXIn vivomedicineAnimalsHumansVibrioToxinEndothelial Cellsbiology.organism_classificationmedicine.diseaseVirologyVibrioinfectionDisease Models Animal030104 developmental biologyInfecciógene expressionCytokine stormEx vivoFrontiers in Cellular and Infection Microbiology
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Unexpected Bacterial Origin of the Antibiotic Icosalide: Two-Tailed Depsipeptide Assembly in Multifarious Burkholderia Symbionts.

2018

Icosalide is an unusual two-tailed lipocyclopeptide antibiotic that was originally isolated from a fungal culture. Yet, its biosynthesis and ecological function have remained enigmatic. By genome mining and metabolic profiling of a bacterial endosymbiont ( Burkholderia gladioli) of the pest beetle Lagria villosa, we unveiled a bacterial origin of icosalide. Functional analysis of the biosynthetic gene locus revealed an unprecedented nonribosomal peptide synthetase (NRPS) that incorporates two β-hydroxy acids by means of two starter condensation domains in different modules. This unusual assembly line, which may inspire new synthetic biology approaches, is widespread among many symbiotic Bur…

0301 basic medicinemedicine.drug_classBurkholderia030106 microbiologyAntibioticsBiochemistryGenomePeptides Cyclic03 medical and health sciencesGene clustermedicineAnimalsPeptide SynthasesSymbiosisGeneticsDepsipeptidebiologyPseudomonasGeneral Medicinebiology.organism_classificationAnti-Bacterial AgentsColeoptera030104 developmental biologyBurkholderiaGenes BacterialMolecular MedicineIdentification (biology)ACS chemical biology
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