Search results for "SUBUNITS"

showing 10 items of 120 documents

Autosomal-Recessive Mutations in AP3B2, Adaptor-Related Protein Complex 3 Beta 2 Subunit, Cause an Early-Onset Epileptic Encephalopathy with Optic At…

2016

International audience; Early-onset epileptic encephalopathy (EOEE) represents a heterogeneous group of severe disorders characterized by seizures, interictal epileptiform activity with a disorganized electroencephalography background, developmental regression or retardation, and onset before 1 year of age. Among a cohort of 57 individuals with epileptic encephalopathy, we ascertained two unrelated affected individuals with EOEE associated with developmental impairment and autosomal-recessive variants in AP3B2 by means of whole-exome sequencing. The targeted sequencing of AP3B2 in 86 unrelated individuals with EOEE led to the identification of an additional family. We gathered five addition…

0301 basic medicineMaleMicrocephalyDevelopmental DisabilitiesPostnatal microcephalycopper-metabolismEpilepsy0302 clinical medicineexpansionhermansky-pudlak-syndromeddc:576.5Age of OnsetChilddisordersGenetics (clinical)seizuresGeneticsMEDNIK syndromeSyndrome3. Good healthPedigreeintellectual disabilityChild Preschoolmednik syndromeMicrocephalyFemaleDevelopmental regressionAdaptor Protein Complex 3Genes RecessiveBiologyAP3B103 medical and health sciencesAtrophyReport[ SDV.MHEP ] Life Sciences [q-bio]/Human health and pathologyGeneticsmedicineHumansAdaptor Protein Complex beta SubunitsmousediseaseEpilepsyap-4 deficiencyInfant NewbornInfantmedicine.diseaseOptic Atrophy030104 developmental biologyMutationHermansky–Pudlak syndrome030217 neurology & neurosurgery[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
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Dopaminergic-GABAergic interplay and alcohol binge drinking

2019

© 2019 Elsevier Ltd The dopamine D 3 receptor (D 3 R), in the nucleus accumbens (NAc), plays an important role in alcohol reward mechanisms. The major neuronal type within the NAc is the GABAergic medium spiny neuron (MSN), whose activity is regulated by dopaminergic inputs. We previously reported that genetic deletion or pharmacological blockade of D 3 R increases GABA A α6 subunit in the ventral striatum. Here we tested the hypothesis that D 3 R-dependent changes in GABA A α6 subunit in the NAc affect voluntary alcohol intake, by influencing the inhibitory transmission of MSNs. We performed in vivo and ex vivo experiments in D 3 R knockout (D 3 R −/− ) mice and wild type littermates (D 3 …

0301 basic medicineMalemedicine.medical_specialtyDopaminergic-GABAergicSettore BIO/09 - FISIOLOGIAAlpha6 subunit; Dopamine D3 receptor; Ethanol; Furosemide (PubChem CID: 3440); GABA(A)receptor; Nucleus accumbens; Ro 15-4513; Ro 15-4513 (PubChem CID: 5081); SB 277011A (PubChem CID: 75358288)Alpha6 subunitNucleus accumbensMedium spiny neuronInhibitory postsynaptic potentialNucleus AccumbensBinge Drinking03 medical and health sciencesMiceDopamine D3 receptor0302 clinical medicineDopamine receptor D3Internal medicinemedicineAnimalsFurosemide (PubChem CID: 3440)Nucleus accumbenPharmacology & PharmacyRNA MessengerRo 15-4513GABAergic NeuronsSB 277011A (PubChem CID: 75358288).PharmacologyMice KnockoutEthanolGABAA receptorChemistryDopaminergicAntagonistReceptors Dopamine D3Receptors GABA-ARo 15-4513 (PubChem CID: 5081)GABA(A)receptor3. Good healthProtein Subunits030104 developmental biologyEndocrinologynervous systemGene Expression Regulation030220 oncology & carcinogenesisGABAergicNucleus accumbensSB 277011A (PubChem CID: 75358288)
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Aromatic and proteomic analyses corroborate the distinction between Mediterranean landraces and modern varieties of durum wheat

2016

AbstractIn this paper volatile organic compounds (VOCs) from durum wheat cultivars and landraces were analyzed using PTR-TOF-MS. The aim was to characterize the VOC’s profile of the wholemeal flour and of the kernel to find out if any VOCs were specific to varieties and sample matrices. The VOC data is accompanied by SDS-PAGE analyses of the storage proteins (gliadins and glutenins). Statistical analyses was carried out both on the signals obtained by MS and on the protein profiles. The difference between the VOC profile of two cultivars or two preparations of the same sample - matrices, in this case kernel vs wholemeal flour - can be very subtle; the high resolution of PTR-TOF-MS - down to…

0301 basic medicineMediterranean climateVOLATILE COMPOUNDSPTR-TOF-MS; VOLATILE COMPOUNDS; GLUTEN STRENGTH; RAPID CHARACTERIZATION; PROTEIN-COMPOSITION; EXTRUSION-COOKING; QUALITY; CULTIVARS; FLOUR; SUBUNITS02 engineering and technologyPROTEIN-COMPOSITIONGliadinFLOURSettore BIO/04 - Fisiologia VegetaleCultivarFood scienceTriticumMathematicschemistry.chemical_classificationPrincipal Component AnalysisMultidisciplinarybiologyvolatile organic compounds; protein composition; gluten strength; quality; cultivars021001 nanoscience & nanotechnologyWholemeal flourBiological EvolutionSUBUNITSCULTIVARSSettore AGR/02 - Agronomia E Coltivazioni ErbaceeItalyPlant proteinSeedsPrincipal component analysis0210 nano-technologyGLUTEN STRENGTHRAPID CHARACTERIZATIONGlutensArticle03 medical and health sciencesfoodBotanyQUALITYStorage proteinPlant breedingVolatile Organic CompoundsEXTRUSION-COOKINGDurum wheat Volatile Organic Compounds VOC profilefood.foodPlant Breeding030104 developmental biologychemistrySpectrometry Mass Matrix-Assisted Laser Desorption-Ionizationbiology.proteinPTR-TOF-MSGliadinScientific Reports
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Auxiliary α2δ1 and α2δ3 Subunits of Calcium Channels Drive Excitatory and Inhibitory Neuronal Network Development

2020

VGCCs are multisubunit complexes that play a crucial role in neuronal signaling. Auxiliary α2δ subunits of VGCCs modulate trafficking and biophysical properties of the pore-forming α1 subunit and trigger excitatory synaptogenesis. Alterations in the expression level of α2δ subunits were implicated in several syndromes and diseases, including chronic neuropathic pain, autism, and epilepsy. However, the contribution of distinct α2δ subunits to excitatory/inhibitory imbalance and aberrant network connectivity characteristic for these pathologic conditions remains unclear. Here, we show that α2δ1 overexpression enhances spontaneous neuronal network activity in developing and mature cultures of …

0301 basic medicineNeurogenesisSynaptogenesisNeurotransmissionInhibitory postsynaptic potentialHippocampusSynaptic Transmission03 medical and health sciencesGlutamatergicMice0302 clinical medicineVGCCsexcitation to inhibition balanceBiological neural networkPremovement neuronal activityAnimalsHumansCalcium SignalingResearch ArticlesNeuronssynaptogenesisChemistryGeneral NeuroscienceGlutamate receptornetwork connectivityRats030104 developmental biologyHEK293 CellsExcitatory postsynaptic potentialalpha2delta subunitsCalcium ChannelsNerve NetNeuroscience030217 neurology & neurosurgeryCellular/MolecularThe Journal of Neuroscience
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DNA methylation links prenatal smoking exposure to later life health outcomes in offspring

2019

Background Maternal smoking during pregnancy is associated with adverse offspring health outcomes across their life course. We hypothesize that DNA methylation is a potential mediator of this relationship. Methods We examined the association of prenatal maternal smoking with offspring blood DNA methylation in 2821 individuals (age 16 to 48 years) from five prospective birth cohort studies and perform Mendelian randomization and mediation analyses to assess whether methylation markers have causal effects on disease outcomes in the offspring. Results We identify 69 differentially methylated CpGs in 36 genomic regions (P value < 1 × 10−7) associated with exposure to maternal smoking in adolesc…

0301 basic medicinePhysiologyraskausDiseaseBioinformaticsEpigenesis Genetic/dk/atira/pure/core/keywords/icepCohort Studies0302 clinical medicinePregnancyGTP-Binding Protein gamma SubunitsEpidemiologySCHIZOPHRENIADiseaseLongitudinal StudiesProspective StudieskohorttitutkimusGenetics (clinical)Maternal smokingGenetics & HeredityRISK0303 health sciencesDNA methylationSmokingWIDEMethylationASSOCIATIONMiddle AgedDNA-metylaatio3. Good healthCausalityPREGNANCYOncologyMaternal ExposureSchizophreniaPrenatal Exposure Delayed Effects030220 oncology & carcinogenesisDNA methylationkausaliteettilifecourseLife course approachFemaleICEPLife Sciences & BiomedicineAdultTOBACCO-SMOKEMediation (statistics)medicine.medical_specialtyAdolescentOffspringBirth weightPersistenceYoung Adult03 medical and health sciencestupakointiterveysvaikutuksetMendelian randomizationGeneticsmedicineHumansMolecular BiologyMETAANALYSIS030304 developmental biologyPregnancyScience & TechnologyIDENTIFICATIONbusiness.industryMATERNAL CIGARETTE-SMOKINGResearchMediationLife courseMendelian Randomization Analysismedicine.diseaseBIRTH-WEIGHT030104 developmental biologyCpG Islandsbusiness030217 neurology & neurosurgeryGenome-Wide Association StudyDevelopmental Biology
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Triclosan-Evoked Neurotoxicity Involves NMDAR Subunits with the Specific Role of GluN2A in Caspase-3-Dependent Apoptosis

2018

Triclosan (TCS) is an antimicrobial agent that is used extensively in personal care and in sanitising products. A number of studies have shown the presence of TCS in different human tissues such as blood, adipose tissue, the liver, brain as well as in breast milk and urine. N-Methyl-D-aspartate receptors (NMDARs) are glutamate-gated ion channels that are widely expressed in the central nervous system and which play key roles in excitatory synaptic transmission. There is, however, no data on the involvement of NMDAR subunits in the apoptotic and neurotoxic effects of TCS. Our experiments are the first to show that TCS used at environmentally relevant concentrations evoked NMDA-dependent effe…

0301 basic medicineProgrammed cell deathGluN1Protein subunitNeurotoxinsNeuroscience (miscellaneous)Glutamic AcidCaspase 3ApoptosisReceptors N-Methyl-D-AspartateArticle03 medical and health sciencesCellular and Molecular NeuroscienceMice0302 clinical medicinemedicineAnimalsGene SilencingRNA MessengerReceptorNeuronsL-Lactate DehydrogenaseChemistryCaspase 3fungiNeurotoxicityROSTransfectionmedicine.diseaseTriclosanCell biologyGluN2BGluN2AProtein Subunits030104 developmental biologyNeurologyNMDAApoptosisNMDA receptorFemale030217 neurology & neurosurgeryMolecular Neurobiology
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A protein-RNA interaction atlas of the ribosome biogenesis factor AATF

2018

AbstractAATF is a central regulator of the cellular outcome upon p53 activation, a finding that has primarily been attributed to its function as a transcription factor. Recent data showed that AATF is essential for ribosome biogenesis and plays a role in rRNA maturation. AATF has been implicated to fulfil this role through direct interaction with rRNA and was identified in several RNA-interactome capture experiments. Here, we provide a first comprehensive analysis of the RNA bound by AATF using CLIP-sequencing. Interestingly, this approach shows predominant binding of the 45S pre-ribosomal RNA precursor molecules. Furthermore, AATF binds to mRNAs encoding for ribosome biogenesis factors as …

0301 basic medicineRibosomal ProteinsRegulatorRibosome biogenesisProteomic analysislcsh:MedicineInteractomeArticleCell Line03 medical and health sciencesMice0302 clinical medicineRNA PrecursorsAnimalsHumansSmall nucleolar RNABinding sitelcsh:ScienceTranscription factor030304 developmental biologyRNA metabolism0303 health sciencesMultidisciplinaryBinding SitesChemistrylcsh:RRNARibosomal RNACell biologyRibosome Subunits SmallRepressor Proteins030104 developmental biologyHEK293 Cells030220 oncology & carcinogenesisRNAlcsh:QApoptosis Regulatory ProteinsRibosomes030217 neurology & neurosurgeryProtein BindingScientific Reports
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Membrane insertion and topology of the translocon-associated protein (TRAP) gamma subunit

2017

Translocon-associated protein (TRAP) complex is intimately associated with the ER translocon for the insertion or translocation of newly synthesised proteins in eukaryotic cells. The TRAP complex is comprised of three single-spanning and one multiple-spanning subunits. We have investigated the membrane insertion and topology of the multiple-spanning TRAP-γ subunit by glycosylation mapping and green fluorescent protein fusions both in vitro and in cell cultures. Results demonstrate that TRAP-γ has four transmembrane (TM) segments, an Nt/Ct cytosolic orientation and that the less hydrophobic TM segment inserts efficiently into the membrane only in the cellular context of full-length protein.

0301 basic medicineVesicle-associated membrane protein 8Receptors PeptideProtein subunitBiophysicsReceptors Cytoplasmic and NuclearBiologyEndoplasmic ReticulumTopologyBiochemistryGreen fluorescent protein03 medical and health sciencesN-linked glycosylationMembranes (Biologia)Membrane GlycoproteinsEndoplasmic reticulumCalcium-Binding ProteinsProteïnes de membranaMembrane ProteinsCell BiologyTransloconTransmembrane proteinProtein Subunits030104 developmental biologyHydrophobic and Hydrophilic InteractionsGamma subunit
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More than a pore: How voltage-gated calcium channels act on different levels of neuronal communication regulation.

2021

ABSTRACT Voltage-gated calcium channels (VGCCs) represent key regulators of the calcium influx through the plasma membrane of excitable cells, like neurons. Activated by the depolarization of the membrane, the opening of VGCCs induces very transient and local changes in the intracellular calcium concentration, known as calcium nanodomains, that in turn trigger calcium-dependent signaling cascades and the release of chemical neurotransmitters. Based on their central importance as concierges of excitation-secretion coupling and therefore neuronal communication, VGCCs have been studied in multiple aspects of neuronal function and malfunction. However, studies on molecular interaction partners …

0301 basic medicineα2δ subunitsBiophysicschemistry.chemical_elementReviewNeurotransmissionCalciumBiochemistrySynaptic TransmissionCalcium in biology03 medical and health sciencesvoltage-induced calcium releasealternative splicing0302 clinical medicinevoltage-gated calcium channelsCavβ subunitsVGCC auxiliary subunitsCalcium SignalingIon channelNeuronssynaptogenesisVoltage-dependent calcium channelChemistryRyanodine receptorDepolarization030104 developmental biologyIon channelsCalciumgene regulationNeuroscience030217 neurology & neurosurgeryIntracellularResearch ArticleChannels (Austin, Tex.)
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Conversion of the Ca2+-ATPase from Rhodospirillum rubrum into a Mg2+-dependent enzyme by 1,N6-etheno ATP

1980

Nucleoside triphosphate hydrolysis of R.rubrum ATPase complexes can be changed from Ca2+-dependence to Mg2+-dependence by replacing ATP with 1,N6-etheno ATP. Four ATPase complexes which have been prepared by different procedures hydrolyze ATP and 1,N6-etheno ATP at different rates in dependence on the added metal ions. These differences allow an easy distinction of the various enzyme forms.

ATPaseBiophysicsPhotophosphorylationCalcium-Transporting ATPasesRhodospirillum rubrumBiochemistrychemistry.chemical_compoundAdenosine TriphosphateMagnesiumMolecular BiologyEdetic Acidchemistry.chemical_classificationbiologyATP synthaseChemiosmosisCell MembraneRhodospirillum rubrumCell Biologybiology.organism_classificationKineticsEnzymeBiochemistrychemistrybiology.proteinNucleoside triphosphateOligomycinsATP synthase alpha/beta subunitsEthenoadenosine TriphosphateProtein BindingBiochemical and Biophysical Research Communications
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