Search results for "GTPase"

showing 10 items of 83 documents

Neuronal Cytoskeleton in Intellectual Disability: From Systems Biology and Modeling to Therapeutic Opportunities

2021

Intellectual disability (ID) is a pathological condition characterized by limited intellectual functioning and adaptive behaviors. It affects 1–3% of the worldwide population, and no pharmacological therapies are currently available. More than 1000 genes have been found mutated in ID patients pointing out that, despite the common phenotype, the genetic bases are highly heterogeneous and apparently unrelated. Bibliomic analysis reveals that ID genes converge onto a few biological modules, including cytoskeleton dynamics, whose regulation depends on Rho GTPases transduction. Genetic variants exert their effects at different levels in a hierarchical arrangement, starting from the molecular lev…

0301 basic medicineactin cytoskeletonReview0302 clinical medicineBorderline intellectual functioningIntellectual disabilityDisabilità Intellettiva GTPasi CitoscheletroBiology (General)CytoskeletonSpectroscopyNeuronseducation.field_of_studysystems biologyCognitionGeneral MedicinePhenotypeComputer Science ApplicationsChemistryPhenotypeintellectual disabilitySignal TransductionBoolean modelingQH301-705.5NeurogenesisIn silicoSystems biologyPopulationBiologyCatalysismicrotubulesInorganic Chemistry03 medical and health sciencesmedicineAnimalsHumansPhysical and Theoretical ChemistryeducationQD1-999Molecular BiologyGTPase signalingsmall Rho GTPasesOrganic Chemistrypharmacological modulationprotein:protein interaction networkActin cytoskeletonmedicine.disease030104 developmental biologySynapsesneuronal networksNeuroscience030217 neurology & neurosurgery
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Host Cell Rab GTPases in Hepatitis B Virus Infection

2018

Hepatitis B virus (HBV) is a leading cause of liver disease and is presently estimated to infect more than 250 million humans. The extremely successful spread of this virus among the human population is explained by its effective transmission strategies and its manifold particle types, including virions, empty envelopes and naked capsids. Due to its tiny genome, HBV depends on cellular machineries to thrive in infected hepatocytes. To enter, traverse and exit the cell, HBV exploits host membrane trafficking pathways, including intracellular highways directed by Rab GTPases. Here, we review recent discoveries focused on how HBV co-opts and perturbs host Rab GTPase functions with an emphasis …

0301 basic medicineautophagyPopulationvirus assemblyReviewGTPaseBiologymedicine.disease_causeVirusRab33BCell and Developmental Biology03 medical and health sciencesViral life cyclemedicineHBVeducationlcsh:QH301-705.5Hepatitis B viruseducation.field_of_studyRab effector030102 biochemistry & molecular biologyEffectorCell BiologyRab7ARab GAPCell biology030104 developmental biologyRAB7Avirus traffickinglcsh:Biology (General)RabDevelopmental BiologyFrontiers in Cell and Developmental Biology
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The integration of autophagy and cellular trafficking pathways via RAB GAPs.

2015

Macroautophagy is a conserved degradative pathway in which a double-membrane compartment sequesters cytoplasmic cargo and delivers the contents to lysosomes for degradation. Efficient formation and maturation of autophagic vesicles, so-called phagophores that are precursors to autophagosomes, and their subsequent trafficking to lysosomes relies on the activity of small RAB GTPases, which are essential factors of cellular vesicle transport systems. The activity of RAB GTPases is coordinated by upstream factors, which include guanine nucleotide exchange factors (RAB GEFs) and RAB GTPase activating proteins (RAB GAPs). A role in macroautophagy regulation for different TRE2-BUB2-CDC16 (TBC) dom…

0301 basic medicineautophagyRAB GTPaseGTPase-activating proteinGTPaseBiologyRAB GAP03 medical and health sciences0302 clinical medicineAnimalsGuanine Nucleotide Exchange FactorsHumansRAB3GAPMolecular Biologyautophagosome formationVesicleAutophagyCellular VesiclefungiGTPase-Activating ProteinsView and CommentaryCell BiologyTransport proteinCell biologyProtein Transport030104 developmental biologyrab GTP-Binding Proteinsvesicle traffickingGuanine nucleotide exchange factorRabLysosomes030217 neurology & neurosurgeryAutophagy
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Uncoupling of dynamin polymerization and GTPase activity revealed by the conformation-specific nanobody dynab

2017

Dynamin is a large GTPase that forms a helical collar at the neck of endocytic pits, and catalyzes membrane fission (Schmid and Frolov, 2011; Ferguson and De Camilli, 2012). Dynamin fission reaction is strictly dependent on GTP hydrolysis, but how fission is mediated is still debated (Antonny et al., 2016): GTP energy could be spent in membrane constriction required for fission, or in disassembly of the dynamin polymer to trigger fission. To follow dynamin GTP hydrolysis at endocytic pits, we generated a conformation-specific nanobody called dynab, that binds preferentially to the GTP hydrolytic state of dynamin-1. Dynab allowed us to follow the GTPase activity of dynamin-1 in real-time. We…

0301 basic medicineendocrine systemGTP'MouseQH301-705.5FissionScienceEndocytic cycleGTPasemacromolecular substancesEndocytosisGeneral Biochemistry Genetics and Molecular BiologyGTP PhosphohydrolasesPolymerization03 medical and health sciences0302 clinical medicineMembrane fissiondynaminendocytosisHumansBiology (General)Dynamin IDynaminGeneral Immunology and MicrobiologyChemistryGeneral Neuroscienceconformational-specific nanobodyHydrolysisQRGeneral MedicineCell BiologyFibroblastsSingle-Domain Antibodiesenzyme030104 developmental biologyMembraneddc:540BiophysicsMedicineGuanosine Triphosphatebiological phenomena cell phenomena and immunitycell biology conformational-specific nanobody dynamin endocytosis enzyme human mouse030217 neurology & neurosurgeryResearch ArticleHumaneLife
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1H, 13C, and 15N NMR chemical shift assignment of the complex formed by the first EPEC EspF repeat and N-WASP GTPase binding domain

2021

AbstractLEE-encoded effector EspF (EspF) is an effector protein part of enteropathogenic Escherichia coli’s (EPEC’s) arsenal for intestinal infection. This intrinsically disordered protein contains three highly conserved repeats which together compose over half of the protein’s complete amino acid sequence. EPEC uses EspF to hijack host proteins in order to promote infection. In the attack EspF is translocated, together with other effector proteins, to host cell via type III secretion system. Inside host EspF stimulates actin polymerization by interacting with Neural Wiskott-Aldrich syndrome protein (N-WASP), a regulator in actin polymerization machinery. It is presumed that EspF acts by di…

030303 biophysicsRegulatormacromolecular substancesBiochemistryArticleType three secretion system03 medical and health sciencesStructural BiologyEnteropathogenic Escherichia coliNMR-spektroskopiaN-WASPPeptide sequenceActin030304 developmental biologysolution NMRSolution NMR0303 health sciencesEffectorChemistryResonance assignmentsresonance assignmentsNuclear magnetic resonance spectroscopyintrinsically disordered protein3. Good healthCell biologytype III secretion systemType III secretion systemIntrinsically disordered proteinEPEC EspFproteiinitGTPase bindingBiomolecular Nmr Assignments
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Azathioprine suppresses ezrin-radixin-moesin-dependent T cell-APC conjugation through inhibition of Vav guanosine exchange activity on rac proteins

2006

Abstract We have shown recently that the azathioprine metabolite 6-Thio-GTP causes immunosuppression by blockade of GTPase activation in T lymphocytes. In the present study, we describe a new molecular mechanism by which 6-Thio-GTP blocks GTPase activation. Although 6-Thio-GTP could bind to various small GTPases, it specifically blocked activation of Rac1 and Rac2 but not of closely related Rho family members such as Cdc42 and RhoA in primary T cells upon stimulation with αCD28 or fibronectin. Binding of 6-Thio-GTP to Rac1 did not suppress Rac effector coupling directly but blocked Vav1 exchange activity upon 6-Thio-GTP hydrolysis, suggesting that 6-Thio-GTP loading leads to accumulation of…

AdultCD4-Positive T-LymphocytesVAV1RHOAT cellImmunologyBlotting WesternAntigen-Presenting CellsFluorescent Antibody TechniqueRAC1ApoptosisEnzyme-Linked Immunosorbent AssayGTPaseCell CommunicationBiologyArticleAzathioprinemedicineImmunology and AllergyHumansAntigen-presenting cellProto-Oncogene Proteins c-vavNeurofibromin 2Flow CytometryMolecular biologyCell biologyrac GTP-Binding ProteinsRac GTP-Binding ProteinsEnzyme Activationmedicine.anatomical_structurebiology.proteinSignal transductionImmunosuppressive Agents
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Oligophrenin 1 mutations frequently cause X-linked mental retardation with cerebellar hypoplasia

2005

Background: Mutations of oligophrenin 1, one of the first genes identified in nonspecific X-linked mental retardation (MRX), have been described in patients with moderate to severe cognitive impairment and predominant cerebellar hypoplasia, in the vermis. Objective: To further delineate the phenotypic and mutational spectrum of the syndrome, by screening oligophrenin 1 in two cohorts of male patients with mental retardation (MR) with or without known posterior fossa anomalies. Methods: Clinical examination, cognitive testing, MRI studies, and mutational analysis (denaturing gradient gel electrophoresis and direct sequencing) on blood lymphocytes were performed in 213 unrelated affected indi…

AdultMaleCerebellumAdolescentGenotypeDNA Mutational AnalysisNonsense mutationNervous System Malformationsmedicine.disease_causeCohort StudiesExonCerebellar DiseasesCerebellummedicineHumansGenetic TestingChildCerebellar hypoplasiaGeneticsMutationSplice site mutationGTPase-Activating ProteinsNuclear Proteinsmedicine.diseaseMagnetic Resonance ImagingHypoplasiaPedigreeDevelopmental disorderAlternative SplicingCytoskeletal ProteinsPhenotypemedicine.anatomical_structureFacial AsymmetryCodon NonsenseChild PreschoolMutationMental Retardation X-LinkedRNA Splice SitesNeurology (clinical)PsychologyGene DeletionNeurology
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A genome-wide association study of monozygotic twin-pairs suggests a locus related to variability of serum high-density lipoprotein cholesterol

2012

Serum lipid levels have been associated with cardiovascular diseases, metabolic syndrome and type II diabetes (Kannel et al., 1961; Miller & Miller, 1975; Pilia et al., 2006). Variation in lipids levels is highly influenced by heritable factors (Friedlander et al., 1997) and 95 loci have already been associated with levels of high density lipoprotein (HDL) cholesterol, low density lipoprotein (LDL) cholesterol, triglycerides (TG) and total cholesterol (TC) in numerous study samples and replicated in various populations using genome-wide approaches (Aulchenko et al., 2008; Kathiresan et al., 2008; Kooner et al., 2008; Teslovich et al., 2010; Willer et al., 2008). However, the genetic associa…

AdultNetherlands Twin Register (NTR)Candidate genegenetiikkaPopulationkolesteroliMonozygotic twinLocus (genetics)Genome-wide association studyBiologyPolymorphism Single NucleotideArticle03 medical and health sciences0302 clinical medicineGenetic variation/dk/atira/pure/keywords/cohort_studies/netherlands_twin_register_ntr_HumansAlleleGene–environment interactioneducationAllelesGenetics (clinical)Aged030304 developmental biologyAged 80 and overGenetics0303 health scienceseducation.field_of_studyperinnöllisyystiedeCholesterol HDLGTPase-Activating ProteinsObstetrics and Gynecologyta3141Twins MonozygoticMiddle AgedIntrons3. Good healthGenetic LociPediatrics Perinatology and Child HealthFemaleGene-Environment InteractionApolipoprotein A-II030217 neurology & neurosurgeryGenome-Wide Association Study
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Downregulation of RhoB GTPase confers resistance to cisplatin in human larygneal carcinoma cells

2009

Acquired resistance to cisplatin represents a major obstacle to an efficient chemotherapy. We found downregulation of RhoB expression in cisplatin-resistant tumor cell lines from different origin. In cisplatin-resistant laryngeal carcinoma subline overexpression of farnesylated or geranylgeranylated RhoB increased cisplatin-induced cell death, while silencing of RhoB expression diminished sensitivity of parental HEp-2 cells via decreased cellular accumulation of cisplatin. However, since RhoB silencing in additional tumor cell lines did not alter their sensitivity to cisplatin, we can assume that RhoB downregulation does not provide general protective role in cell response to cisplatin. Nev…

Cancer ResearchProgrammed cell deathGenetic enhancementRHOBmedicine.medical_treatmentDown-RegulationAntineoplastic AgentsBiologyDownregulation and upregulationCell Line TumormedicineCarcinomaHumansGene silencingrhoB GTP-Binding ProteinLaryngeal NeoplasmsCisplatinChemotherapymedicine.diseaseCell biologyOncologyDrug Resistance NeoplasmCancer researchcisplatin ; drug resistance ; Rho GTPases ; RhoBCisplatinmedicine.drug
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Inhibitors of Rho-kinase modulate amyloid-β (Aβ) secretion but lack selectivity for Aβ42

2005

Certain non-steroidal anti-inflammatory drugs (NSAIDs) preferentially inhibit production of the amyloidogenic Abeta42 peptide, presumably by direct modulation of gamma-secretase activity. A recent report indicated that NSAIDs could reduce Abeta42 by inhibition of the small GTPase Rho, and a single inhibitor of Rho kinase (ROCK) mimicked the effects of Abeta42-lowering NSAIDs. To investigate whether Abeta42 reduction is a common property of ROCK inhibitors, we tested commercially available compounds in cell lines that were previously used to demonstrate the Abeta42-lowering activity of NSAIDs. Surprisingly, we found that two ROCK inhibitors reduced total Abeta secretion in a dose-dependent m…

Cell SurvivalMutantPeptideCHO CellsProtein Serine-Threonine KinasesPharmacologyBiochemistryAmyloid beta-Protein PrecursorCellular and Molecular NeuroscienceCricetulusCricetinaeEndopeptidasesmental disordersAmyloid precursor proteinAnimalsAspartic Acid EndopeptidasesSecretionSmall GTPaseEnzyme InhibitorsRho-associated protein kinasechemistry.chemical_classificationrho-Associated KinasesAmyloid beta-PeptidesbiologyAnti-Inflammatory Agents Non-SteroidalIntracellular Signaling Peptides and ProteinsIn vitro toxicologyProtein-Tyrosine KinasesPeptide Fragmentsnervous system diseasesBiochemistrychemistrybiology.proteinAmyloid Precursor Protein SecretasesSelectivityProtein Processing Post-TranslationalJournal of Neurochemistry
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