Search results for "RAM"

showing 10 items of 35643 documents

Apoptosis-related Proteins in Skeletal Muscle Fibers of Spinal Muscular Atrophy

1997

There is evidence that apoptosis in spinal muscular atrophies (SMA) is not restricted to motor neurons but also affects muscle fibers. Studying the expression of several apoptosis-associated proteins we found constant expression of bax in muscle fibers, which promoted cell death. The expression of bax correlated with defective innervation of muscle fibers was also indicated by upregulation of N-CAM. While in early-onset SMA atrophic as well as normo- and hypertrophic muscle fibers displayed expression of bax, muscle fibers in late-onset SMA and peripheral neuropathies showed bax-expression only in atrophic fibers. Other investigated apoptosis-associated factors comprised interleukin-1 beta …

AdultMaleProgrammed cell deathPathologymedicine.medical_specialtyMuscle Fibers Skeletalbcl-X ProteinMuscle ProteinsApoptosisBiologyMicrofilamentPathology and Forensic MedicineMuscular Atrophy SpinalCellular and Molecular NeuroscienceReference ValuesProto-Oncogene ProteinsmedicineHumansMyocyteMuscle SkeletalActinAgedbcl-2-Associated X ProteinCaspase 1InfantPeripheral Nervous System DiseasesGeneral MedicineSpinal muscular atrophyMiddle AgedSMA*Spinal muscular atrophiesmedicine.diseaseCell biologyCysteine EndopeptidasesProto-Oncogene Proteins c-bcl-2NeurologyFemaleNeural cell adhesion moleculeNeurology (clinical)Journal of Neuropathology and Experimental Neurology
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De novo mutation in a male patient with Fabry disease: a case report

2014

Abstract Background Fabry disease is an X-linked inherited metabolic condition where the deficit of the α-galactosidase A enzyme, encoded by the GLA gene, leads to glycosphingolipid storage, mainly globotriaosylceramide. To date, more than 600 mutations have been identified in human GLA gene that are responsible for FD, including missense and nonsense mutations, small and large deletions. Such mutations are usually inherited, and cases of de novo onset occur rarely. Case presentation In this article we report an interesting case of a 44-year-old male patient suffering from a severe form of Fabry disease, with negative family history. The patient showed signs such as cornea verticillata, ang…

AdultMaleProtein Foldingα-galactosidase ADe novo mutationNonsense mutationD165H mutationGlobotriaosylceramideMutation MissenseCase ReportBiologymedicine.disease_causeGeneral Biochemistry Genetics and Molecular Biologychemistry.chemical_compoundGermline mutationmedicineMissense mutationHumansPoint MutationThrombophiliaEnzyme Replacement TherapyAmino Acid SequenceChildGLA geneConserved SequenceGerm-Line MutationMedicine(all)GeneticsMutationFabry diseaseSequence Homology Amino AcidBiochemistry Genetics and Molecular Biology(all)Point mutationGeneral MedicineEnzyme replacement therapymedicine.diseaseFabry diseasePedigreeStrokechemistryAmino Acid Substitutionalpha-GalactosidaseKidney Failure ChronicFemaleSymptom AssessmentSequence AlignmentBMC Research Notes
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Proteomics of tear fluid in thyroid-associated orbitopathy.

2012

Proteomics and mass spectrometry are useful tools for peptide screening in body fluids. In thyroid-associated orbitopathy (TAO), evidence for lacrimal gland involvement with altered composition of tears has been reported. Our objective was to detect and evaluate potential changes in the proteomic patterns of tear fluid in TAO.Tear fluid was collected from 45 patients with TAO and 15 healthy controls. Tear proteins were analyzed using surface-enhanced laser desorption/ionization time-of-flight mass spectrometry, and peptides were identified using matrix-assisted laser desorption/ionization time-of-flight technology.Peptides with molecular weights 3808 Dalton (Da, p=0.004), 3734 Da (p=0.034),…

AdultMaleProteomicsmedicine.medical_specialtyEndocrinology Diabetes and MetabolismDown-RegulationPeptideLacrimal glandProteomicsMass spectrometryEndocrinologyInternal medicinemedicineHumansEye ProteinsAgedchemistry.chemical_classificationMolecular massThyroidLacrimal ApparatusMiddle AgedUp-RegulationGraves OphthalmopathyMatrix-assisted laser desorption/ionizationmedicine.anatomical_structureEndocrinologychemistrySpectrometry Mass Matrix-Assisted Laser Desorption-IonizationTearsTearsSalivary CystatinsFemaleMuramidasePeptidesbeta 2-MicroglobulinThyroid : official journal of the American Thyroid Association
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Erythema nodosum-like lesions during BRAF inhibitor therapy: Report on 16 new cases and review of the literature.

2015

Importance BRAF inhibitors have been licensed for the therapy of BRAF-mutated melanoma. Recently, inflammatory skin lesions clinically resembling erythema nodosum have been reported as therapy side-effects that may lead to treatment discontinuation. Objective To identify and characterize cases with BRAF inhibitor-associated erythema nodosum-like inflammatory skin lesions and development of an algorithm for their management. Design and Setting Retrospective chart review of melanoma patients treated with BRAF inhibitors in 14 departments of Dermatology in Germany and Austria and PubMed search for cases in the literature. Results Sixteen patients were identified who developed erythema nodosum-…

AdultMaleProto-Oncogene Proteins B-rafmedicine.medical_specialtyIndolesErythemaBiopsyMedizinDermatology030207 dermatology & venereal diseases03 medical and health sciencesYoung Adult0302 clinical medicineErythema NodosumOximesmedicineHumansskin and connective tissue diseasesVemurafenibneoplasmsAgedRetrospective StudiesSkinTrametinibErythema nodosumSulfonamidesintegumentary systembusiness.industryMelanomaImidazolesDabrafenibMiddle Agedmedicine.diseaseDermatology3. Good healthInfectious DiseasesVemurafenib030220 oncology & carcinogenesisFemalemedicine.symptombusinessPanniculitisVasculitismedicine.drugJournal of the European Academy of Dermatology and Venereology : JEADV
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Liebowitz Social Anxiety Scale (LSAS): Optimal cut points for remission and response in a German sample

2017

The Liebowitz Social Anxiety Scale (LSAS) is the most frequently used instrument to assess social anxiety disorder (SAD) in clinical research and practice. Both a self-reported (LSAS-SR) and a clinician-administered (LSAS-CA) version are available. The aim of the present study was to define optimal cut-off (OC) scores for remission and response to treatment for the LSAS in a German sample. Data of N = 311 patients with SAD were used who had completed psychotherapeutic treatment within a multicentre randomized controlled trial. Diagnosis of SAD and reduction in symptom severity according to the Structured Clinical Interview for Diagnostic and Statistical Manual of Mental Disorders, 4th editi…

AdultMalePsychometricsLiebowitz social anxiety scaleSensitivity and Specificitylaw.invention03 medical and health sciences0302 clinical medicineRandomized controlled triallawGermanyHumansPsychiatric Status Rating ScalesReceiver operating characteristicRemission InductionSocial anxietyNonparametric statisticsReproducibility of ResultsPhobia SocialGold standard (test)030227 psychiatryPsychotherapyClinical PsychologyTreatment OutcomeClinical researchFemalePsychology030217 neurology & neurosurgeryCut-pointClinical psychologyClinical Psychology & Psychotherapy
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Association between affective temperaments and season of birth in a general student population.

2011

Abstract Background Several studies indicate a significant association between birth season and personality and neuropsychiatric disorders. The aim of our present study was to investigate the association between affective temperaments and season of birth in a nonclinical sample. Methods 366 university students completed the standardized Hungarian version of the Temperament Evaluation of Memphis, Pisa, Paris and San Diego-Auto-questionnaire (TEMPS-A). Ordinary Least Squares regression was applied to explain the relationship between TEMPS-A subscale and birth season of the respondents. Results We found a significant association between temperament scores and birth season in the case of the Hy…

AdultMalePsychometricsSeason of birthAdolescentPersonality InventoryPsychometricsmedia_common.quotation_subjectAffect (psychology)Developmental psychologyYoung AdultSex FactorsPersonalityHumansYoung adultAssociation (psychology)StudentsTemperamentSubclinical infectionmedia_commonaffective temperamentsseasonalityReproducibility of Resultstemps-a; birth season; affective temperaments; seasonality; personalityPsychiatry and Mental healthClinical PsychologyAffectpersonalityTemperamenttemps-aFemaleSeasonsPsychologybirth seasonJournal of affective disorders
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Facial emotion space in schizophrenia

2007

Introduction. Previous studies show that patients with schizophrenia have a deficit in facial emotion recognition. In the framework of emotion categorisation theories, the purpose of the present study was to test if this impairment could result from abnormal boundaries between emotions (whether these boundaries are shifted along continuums or are less sharpened). Method. Twenty-six schizophrenic patients and the same number of healthy participants were required to perform a facial emotion recognition task and an emotion categorisation task with different emotion intensities obtained using morphing techniques. Results. The main results indicate that schizophrenic patients exhibited an emotio…

AdultMalePsychosisCognitive NeuroscienceSchizophrenia (object-oriented programming)media_common.quotation_subjectSpace (commercial competition)Severity of Illness IndexDelusionsDevelopmental psychologySocial cognitionPerceptionmedicineHumansCategorical variablemedia_commonFacial expressionRecognition Psychologymedicine.diseaseFacial ExpressionAffectPsychiatry and Mental healthCategorizationSchizophreniaFemalePsychologyCognitive psychologyCognitive Neuropsychiatry
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Savoxepine: invalidation of an "atypical" neuroleptic response pattern predicted by animal models in an open clinical trial with schizophrenic patien…

1991

The new tetracyclic compound savoxepine exhibits potent antidopaminergic effects with preferential activity in the hippocampus as compared to striatum in rat brain. As a result of behavioural animal models and regional differences in dopamine receptor binding characteristics, it has been suggested to possess an "atypical" neuroleptic response pattern. In an open clinical trial, savoxepine was administered to 12 in-patients suffering from paranoid schizophrenia and schizophreniform disorder (DSM-III). Eight patients were treated with a stable dose of 0.5 mg per day throughout the study, while in the remaining patients higher doses up to 20 mg/day were administered. Mean total BPRS scores and…

AdultMalePsychosisParanoid schizophreniamedicine.medical_treatmentPharmacologyExtrapyramidal symptomsBasal Ganglia DiseasesmedicineHumansSchizophreniform disorderAntipsychoticAgedPharmacologyPsychiatric Status Rating ScalesDopamine antagonistDopamine receptor bindingMiddle Agedmedicine.diseaseEnzymesDisease Models AnimalSchizophreniaDibenzoxazepinesSchizophreniaFemaleSchizophrenic Psychologymedicine.symptomPsychologymedicine.drugAntipsychotic AgentsPsychopharmacology
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Cognitive impairment is related to oxidative stress and chemokine levels in first psychotic episodes.

2011

Abstract Introduction This study measures the levels of various markers of oxidative stress and inflammation in blood samples from first-episode psychosis (FEP) patients, and examines the association between these peripheral biomarkers and cognitive performance at 6 months after treatment. Methods Twenty-eight FEP patients and 28 healthy controls (matched by age, sex and educational level) had blood samples taken at admission for assessment of total antioxidant status, superoxide dismutase (SOD), total glutathione (GSH), catalase (CAT), glutathione peroxidase, lipid peroxidation, nitrites and the chemokine monocyte chemoattractant protein-1 (MCP-1). A battery of cognitive tests was also app…

AdultMalePsychosismedicine.medical_specialtyNeuropsychological Testsmedicine.disease_causeStatistics NonparametricLipid peroxidationSuperoxide dismutasechemistry.chemical_compoundYoung AdultInternal medicinemedicineHumansBiological PsychiatryChemokine CCL2Nitriteschemistry.chemical_classificationFirst episodePsychiatric Status Rating ScalesGlutathione PeroxidasebiologySuperoxide DismutaseGlutathione peroxidaseCase-control studyGlutathionemedicine.diseaseCatalaseGlutathionePsychiatry and Mental healthOxidative StressEndocrinologychemistryPsychotic DisordersCase-Control StudiesImmunologybiology.proteinLinear ModelsFemaleLipid PeroxidationPsychologyCognition DisordersOxidative stressSchizophrenia research
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Subchronic haloperidol downregulates dopamine synthesis capacity in the brain of schizophrenic patients in vivo

2003

Udgivelsesdato: 2003-Apr The antipsychotic effect of neuroleptics cannot be attributed entirely to acute blockade of postsynaptic D(2)-like dopamine (DA) receptors, but may arise in conjunction with the delayed depolarization block of the presynaptic neurons and reduced DA synthesis capacity. Whereas the phenomenon of depolarization block is well established in animals, it is unknown if a similar phenomenon occurs in humans treated with neuroleptics. We hypothesized that haloperidol treatment should result in decreased DA synthesis capacity. We used 6-[(18)F]fluoro-L-dopa (FDOPA) and positron emission tomography (PET) in conjunction with compartmental modeling to measure the relative activi…

AdultMalePsychosismedicine.medical_specialtyPatientsDopamineDown-RegulationStatistics NonparametricDopamineInternal medicinemedicineHaloperidolHumansPharmacologybusiness.industryPutamenDopamine antagonistBrainDepolarizationHuman brainMiddle Agedmedicine.diseasePsychiatry and Mental healthEndocrinologymedicine.anatomical_structureCerebral cortexAnesthesiaSchizophreniaHaloperidolbusinessTomography Emission-Computedmedicine.drug
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