Search results for "LR"

showing 10 items of 764 documents

Enzyme replacement therapy with recombinant pro-CTSD (cathepsin D) corrects defective proteolysis and autophagy in neuronal ceroid lipofuscinosis

2019

CTSD (cathepsin D) is one of the major lysosomal proteases indispensable for the maintenance of cellular proteostasis by turning over substrates of endocytosis, phagocytosis and autophagy. Consequently, CTSD deficiency leads to a strong impairment of the lysosomal-autophagy machinery. In mice and humans CTSD dysfunction underlies the congenital variant (CLN10) of neuronal ceroid lipofuscinosis (NCL). NCLs are distinct lysosomal storage disorders (LSDs) sharing various hallmarks, namely accumulation of protein aggregates and ceroid lipofuscin leading to neurodegeneration and blindness. The most established and clinically approved approach to treat LSDs is enzyme replacement therapy (ERT) aim…

0301 basic medicineproteolysisCathepsin DCathepsin DCathepsin BstorageCathepsin L03 medical and health sciencesSequestosome 1Neuronal Ceroid-LipofuscinosesAutophagymedicineAnimalsHumansEnzyme Replacement TherapyeducationMolecular BiologyMice Knockouttherapyeducation.field_of_studyTripeptidyl-Peptidase 1030102 biochemistry & molecular biologybiologyAutophagy; cathepsin D; enzyme replacement therapy; lysosome; neuronal ceroid lipofuscinosis; proteolysis; storage; therapyBrainCell BiologyFibroblastsTripeptidyl peptidase Imedicine.diseaseLRP1Cell biologyDisease Models Animal030104 developmental biologylysosomebiology.proteinAllograft inflammatory factor 1Neuronal ceroid lipofuscinosisneuronal ceroid lipofuscinosisLysosomesResearch PaperAutophagy
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Dispersion from Cα or NH: 4D experiments for backbone resonance assignment of intrinsically disordered proteins

2020

AbstractResonance assignment of intrinsically disordered proteins is remarkably challenging due to scant chemical shift dispersion arising from conformational heterogeneity. The challenge is even greater if repeating segments are present in the amino acid sequence. To forward unambiguous resonance assignment of intrinsically disordered proteins, we present iHACANCO, HACACON and (HACA)CONCAHA, three Hα-detected 4D experiments with Cα as an additional dimension. In addition, we present (HACA)CON(CA)NH and (HACA)N(CA)CONH, new 4D Hα-start, HN-detect experiments which have two NH dimensions to enhance peak dispersion in a sequential walk through C′, NH and HN, and provide more accurate NH/HN ch…

0303 health sciencesChemical substanceChemistryChemical shiftIDPintrinsically disordered proteinresonanssi010402 general chemistryIntrinsically disordered proteinsAggregatibacter actinomycetemcomitans01 natural sciencesBiochemistryResonance (particle physics)bakteerit0104 chemical sciences03 medical and health sciencesCrystallographyBilRIproteiinitNMR-spektroskopiaDispersion (chemistry)Peptide sequenceresonance assignmentSpectroscopy030304 developmental biologyJournal of Biomolecular NMR
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2'-O-methylation within prokaryotic and eukaryotic tRNA inhibits innate immune activation by endosomal Toll-like receptors but does not affect recogn…

2019

Bacterial RNA has emerged as an important activator of innate immune responses by stimulating Toll-like receptors TLR7 and TLR8 in humans. Guanosine 2′-O-methylation at position 18 (Gm18) in bacterial tRNA was shown to antagonize tRNA-induced TLR7/8 activation, suggesting a potential role of Gm18 as an immune escape mechanism. This modification also occurs in eukaryotic tRNA, yet a physiological immune function remained to be tested. We therefore set out to investigate the immune modulatory role of Gm18 in both prokaryotic and eukaryotic microorganisms, Escherichia coli and Saccharomyces cerevisiae, and in human cells. Using RiboMethSeq analysis we show that mutation of trmH in E. coli, trm…

0303 health sciencesTRNA modificationInnate immune system030302 biochemistry & molecular biologyRNA[SDV.BBM.BM]Life Sciences [q-bio]/Biochemistry Molecular Biology/Molecular biologyTLR7BiologyTLR8[SDV.BBM.BM] Life Sciences [q-bio]/Biochemistry Molecular Biology/Molecular biologyCell biology03 medical and health sciencesImmune system[SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry Molecular Biology/Genomics [q-bio.GN]Transfer RNAGene expression[SDV.BBM.GTP] Life Sciences [q-bio]/Biochemistry Molecular Biology/Genomics [q-bio.GN]Molecular BiologyComputingMilieux_MISCELLANEOUS030304 developmental biology
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A Process × Domain Assessment of Narcissism: The Domain-Specific Narcissistic Admiration and Rivalry Questionnaire

2022

Research on grandiose narcissism distinguishes between self-promotional processes (i.e., narcissistic admiration) and other-derogative processes (i.e., narcissistic rivalry; Back et al., 2013). Moreover, research has begun to assess and investigate narcissistic manifestations in different domains (e.g., communal narcissism). To integrate these two lines of research, we developed the Domain-Specific Narcissistic Admiration and Rivalry Questionnaire (D-NARQ), a 72-item narcissism questionnaire that contains a self-promotional process scale (narcissistic admiration) and an other-derogatory process scale (narcissistic rivalry) for four domains: intellectual ability, social dominance, communal …

050103 clinical psychologySelf-AssessmentPsychoanalysiscommunionAdmirationPsychometricsphysical attractiveness05 social sciencesPhysical attractiveness050109 social psychologyintelligenceDomain (software engineering)social statusClinical PsychologySurveys and QuestionnairesNarcissismmedicineHumans0501 psychology and cognitive sciencesnarcissismmedicine.symptomPsychologyRivalryApplied Psychology
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Semilunar Granule Cells Are the Primary Source of the Perisomatic Excitatory Innervation onto Parvalbumin-Expressing Interneurons in the Dentate Gyrus

2020

AbstractWe analyzed the origin and relevance of the perisomatic excitatory inputs on the parvalbumin interneurons of the granule cell layer in mouse. Confocal analysis of the glutamatergic innervation showed that it represents ∼50% of the perisomatic synapses that parvalbumin cells receive. This excitatory input may originate from granule cell collaterals, the mossy cells, or even supramammillary nucleus. First, we assessed the input from the mossy cells on parvalbumin interneurons. Axon terminals of mossy cells were visualized by their calretinin content. Using multicolor confocal microscopy, we observed that less than 10% of perisomatic excitatory innervation of parvalbumin cells could or…

6Neuronal ExcitabilityMiceGlutamatergicInterneuronsmedicineAnimalsAxonNeuronselectron microscopybiologyChemistrymusculoskeletal neural and ocular physiologyGeneral NeuroscienceDentate gyrusGeneral MedicinetracingGranule cellAxonsAnterograde tracingParvalbuminsmedicine.anatomical_structurenervous systemDentate GyrusimmunochemistryExcitatory postsynaptic potentialbiology.proteinCalretininNeuroscienceResearch Article: New ResearchmicrocircuitryParvalbumineneuro
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Strong enhancement of superconductivity at high pressures within the charge-density-wave states of 2H-TaS 2 and 2H-TaSe 2

2016

We present measurements of the superconducting and charge density wave critical temperatures (Tc and TCDW) as a function of pressure in the transition metal dichalchogenides 2H-TaSe2 and 2H-TaS2. Resistance and susceptibility measurements show that Tc increases from temperatures below 1 K up to 8.5 K at 9.5 GPa in 2H-TaS2 and 8.2 K at 23 GPa in 2H-TaSe2. We observe a kink in the pressure dependence of TCDW at about 4 GPa that we attribute to the lock-in transition from incommensurate CDW to commensurate CDW. Above this pressure, the commensurate TCDW slowly decreases coexisting with superconductivity within our full pressure range.

7470Xa[PHYS.COND.CM-S]Physics [physics]/Condensed Matter [cond-mat]/Superconductivity [cond-mat.supr-con]Superconductivity (cond-mat.supr-con)Condensed Matter - Strongly Correlated ElectronsStrongly Correlated Electrons (cond-mat.str-el)Condensed Matter - SuperconductivityFOS: Physical sciences7445Lr7425Dw[PHYS.COND.CM-SCE]Physics [physics]/Condensed Matter [cond-mat]/Strongly Correlated Electrons [cond-mat.str-el]
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Estudio del estrés oxidativo hepático asociado a la enfermedad de Alzheimer. Efecto del tratamiento con bexaroteno y/o genisteína

2013

La enfermedad de Alzheimer (EA) no tiene una etiología perfectamente definida. Una de las teorías que se barajan como causa de la EA es la del péptido β-amiloide (βA). Esta molécula es neurotóxica y es capaz de producir estrés oxidativo. Se forma principalmente en el cerebro y se acumula formando placas extracelulares. Se ha sugerido que el βA es el eje principal de la EA, y por tanto que a partir de esta molécula se desencadenan todos los procesos asociados a la EA, lo que se ha llamado la cascada amiloidea. Otra de las hipótesis es la que la relaciona con el estrés oxidativo. Este factor sería el agente que realmente provoca toda la serie de cambios descritos en los tejidos nerviosos, a t…

:CIENCIAS DE LA VIDA::Neurociencias::Neurofisiología [UNESCO]genisteintreatmentLRP-1bexaroteneUNESCO::CIENCIAS DE LA VIDA::Biología animal (Zoología) ::Fisiología animaloxidative stressalzheimer's disease:CIENCIAS DE LA VIDA::Biología animal (Zoología) ::Fisiología animal [UNESCO]liverUNESCO::CIENCIAS DE LA VIDA::Neurociencias::NeurofisiologíaRAGE
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Garantías legales del concepto de privacidad: Entre el derecho al olvido y el nuevo reglamento europeo de protección de datos

2018

The operation of new technologies and the supply of digital goods involves for users to disclose their personal data. The market data is already a reality with the danger that this entails for the privacy of citizens. The new General Data Protection Regulation has created a new legal framework that aims to radically change the current model, giving users greater powers to control their data. A good example of this is the express statement, and for the first time, of the right to be forgotten, which allows citizens to digitally erase their data when other fundamental rights are endangered.

:CIENCIAS JURÍDICAS [UNESCO]2386-4567 22661 Actualidad jurídica iberoamericana 502169 2018 9 6653324 Garantías legales del concepto de privacidad: Entre el derecho al olvido y el nuevo reglamento europeo de protección de datos Sancho LópezprivacidadComputingMilieux_LEGALASPECTSOFCOMPUTINGprivacyBig dataLaw & new technologiesUNESCO::CIENCIAS JURÍDICASMarina The operation of new technologies and the supply of digital goods involves for users to disclose their personal data. The market data is already a reality with the danger that this entails for the privacy of citizens. The new General Data Protection Regulation has created a new legal framework that aims to radically change the current modelright to be forgottenEntre el derecho al olvido y el nuevo reglamento europeo de protección de datos Sancho López [2386-4567 22661 Actualidad jurídica iberoamericana 502169 2018 9 6653324 Garantías legales del concepto de privacidad]Internet.derecho al olvido
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El consentimiento en el proceso penal : ¿un oxímoron?

2021

We are involved in a moment of deep changes in Criminal Procedure due, among other reasons, to the permanent expansion of Criminal Law. Legislative modifications have taken place one another over the world and they have led to a functional reformulation of the role played by the protagonists of Criminal Procedure. These changes have not finished yet and the future is still to come, but new elements are already emerging: the consent of the actors of the process and of the victims and the reinforcement of the principle of opportunity are undoubtedly some of them. Probation, diversion, compliance, criminal mediation are institutions that stand on the principle of consent for procedural purpose…

:CIENCIAS JURÍDICAS [UNESCO]principle of oppotunity 208 235among other reasonscriminal mediation are institutions that stand on the principle of consent for procedural purposes and show this new scenario that is emerging. Speaking about consent in criminal proceedings seems to be no longer an oxymoron Consentimientoto the permanent expansion of Criminal Law. Legislative modifications have taken place one another over the world and they have led to a functional reformulation of the role played by the protagonists of Criminal Procedure. These changes have not finished yet and the future is still to comecompliance2070-8157 22082 Revista Boliviana de Derecho 565487 2021 31 7730057 El consentimiento en el proceso penal ¿un oxímoron? Barona VilarConsentbut new elements are already emerging: the consent of the actors of the process and of the victims and the reinforcement of the principle of opportunity are undoubtedly some of them. ProbationUNESCO::CIENCIAS JURÍDICASdiversionprincipio de oportunidadSilvia We are involved in a moment of deep changes in Criminal Procedure duethe consent of the actors of the process and of the victims and the reinforcement of the principle of opportunity are undoubtedly some of them. Probation [but new elements are already emerging]
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Memorands jaunbūvējamās dzelzceļu līnijas Rīga-Ērgļi-Kārsava posma Ērgļi-Madona lietā

1934

Mašīnraksta kopija

:TECHNOLOGY::Civil engineering and architecture::Building engineering [Research Subject Categories]TransportTransportsDzelzceļa transports - LatvijaRailroads - LatviaDzelzceļa satiksme - LatvijaDzelzceļa līnijas - Latvija
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