Search results for "Epilèpsia"

showing 4 items of 4 documents

Early-infantile onset epilepsy and developmental delay caused by bi-allelic GAD1 variants

2020

Mice lacking GAD1 show neonatal mortality, but the human phenotype associated with GAD1 disruption is poorly characterized. Neuray et al. describe six patients with biallelic GAD1 mutations, presenting with early-infantile onset epilepsy, neurodevelopmental delay, muscle weakness and non-CNS manifestations.

Male0301 basic medicineGlutamate decarboxylaseMalalties cerebralsNeurotransmissorsNeurodevelopmental delayEpilepsy0302 clinical medicineMESH: ChildAge of OnsetChildcleft palateGAD1AcademicSubjects/SCI01870Glutamate DecarboxylaseGlutamate receptorMuscle weakness//purl.org/becyt/ford/3.1 [https]NeurotransmittersMESH: InfantHypotoniamuscle weakneCleft palateMESH: EpilepsyChild PreschoolMuscle Hypotonia[SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]//purl.org/becyt/ford/3 [https]FemaleBrain diseasesAbnormalitiesmedicine.symptomMultiplemedicine.drugcleft palate; epilepsy; GAD1; muscle weakness; neurodevelopmental delayMESH: Glutamate Decarboxylasemedicine.medical_specialtyMESH: Abnormalities MultipleMESH: MutationMESH: Age of OnsetBiologyInhibitory postsynaptic potentialGAD1 cleft palate epilepsy muscle weakness neurodevelopmental delay.gamma-Aminobutyric acidGAD1neurodevelopmental delay03 medical and health sciencesExcitatory synapseInternal medicinemedicineHumansAbnormalities MultiplePreschoolAllelesMESH: Neurodevelopmental Disordersmuscle weaknessMESH: HumansEpilepsyMESH: Muscle HypotoniaMESH: AllelesMESH: Child PreschoolInfantmedicine.diseaseMESH: MaleEpilèpsiaEditor's Choice030104 developmental biologyEndocrinologyNeurodevelopmental DisordersMutationepilepsyAcademicSubjects/MED00310Neurology (clinical)Cleft palate; Epilepsy; GAD1; Muscle weakness; Neurodevelopmental delay; Abnormalities Multiple; Age of Onset; Alleles; Child; Child Preschool; Epilepsy; Female; Glutamate Decarboxylase; Humans; Infant; Male; Muscle Hypotonia; Mutation; Neurodevelopmental DisordersMESH: Female[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology030217 neurology & neurosurgeryReports
researchProduct

α-Synuclein expression levels do not significantly affect proteasome function and expression in mice and stably transfected PC12 cell lines

2004

α-Synuclein (α-syn) is a small protein of unknown function that is found aggregated in Lewy bodies, the histopathological hallmark of sporadic Parkinson disease and other synucleinopathies. Mutations in the α-syn gene and a triplication of its gene locus have been identified in early onset familial Parkinson disease. α-Syn turnover can be mediated by the proteasome pathway. A survey of published data may lead to the suggestion that overexpression of α-syn wild type, and/or their variants (A53T and A30P), may produce a decrease in proteasome activity and function, contributing to α-syn aggregation. To investigate the relationship between synuclein expression and proteasome function we have s…

Time Factorsanimal diseasesmedicine.disease_causePC12 CellsBiochemistryMicechemistry.chemical_compoundTransgenesPromoter Regions GeneticMice KnockoutGeneticsMutationInnervationBrainParkinson DiseaseProteasome complexAmyloidosisCell biologyInnervacióalpha-SynucleinAdditions and CorrectionsPèptidsPlasmidsProteasome Endopeptidase ComplexPrionsProtein subunitBlotting WesternImmunoblottingSynucleinsMice TransgenicNerve Tissue ProteinsBiologyTransfectionBacterial ProteinsMultienzyme ComplexesmedicineAnimalsImmunoprecipitationMolecular BiologyAlpha-synucleinSynucleinopathiesEpilepsyWild typeGenetic VariationCell BiologyAxonsRatsnervous system diseasesMice Inbred C57BLEpilèpsiaDisease Models AnimalLuminescent ProteinschemistryProteasomenervous systemSinapsiMutationSynapsesSynucleinAmiloïdosiPeptides
researchProduct

La literatura médica sobre epilepsia, siglos XVI-XIX: análisis bibliométrico

1976

El objeto del presente trabajo de investigación es el análisis estadístico y sociométrico de las publicaciones sobre epilepsia aparecidas entre los siglos XVI a XIX e indizadas en el Index Catalogue of the Library of the Surgeon-General’s Office. Dos factores han pesado de forma esencial, en la elección del tema: en primer lugar, y tras un vistazo previo a la fuente, la posibilidad de confeccionar un repertorio lo suficientemente abundante para poder realizar el análisis sociométrico. En segundo lugar, la existencia de una monografía sobre la historia de esta enfermedad, clásica en su género. Nos referimos concretamente a la obra de Owsei Temkin The Falling Sickness (Baltimore-London, 2ª ed…

análisis estadísticoanàlisi estadísticaanàlisi sociomètricaanálisis sociométricoUNESCO::HISTORIA::Historia por especialidades::Historia de la medicinaepilèpsiabibliometríahistòriahistoriabibliometriaepilepsiaUNESCO::CIENCIAS MÉDICAS ::Medicina interna::Neurología
researchProduct

Malaltia i salut en l'obra de Francesc Eiximenis

2010

Diversos autors han analitzat els voluminosos tractats de Francesc Eiximenis, especialment pel que fa a matèries relacionades amb la teologia moral, però també sobre altres temes, com la guerra i la pau, l’astrologia, el govern, la cuina i el menjar, i d’altres. En aquest article ens proposem apropar-nos a com Eiximenis tracta el tema dels hospitals, medicina, salut i malaltia. Eiximenis es preocupava de les necessitats del poble que sofria tot tipus de malalties; a part d’haver viscut durant diverses epidèmies, que provocà moltes morts a la península, també suggerí als jurats de València un bon sistema d’hospitals i on s’haurien de construir, i també els cridà l’atenció respecte de les def…

lcsh:Language and LiteratureUNESCO::CIENCIAS DE LAS ARTES Y LAS LETRASLingüísticasalutdietaFilologíasepilèpsiahospitals; malaltia; salut; lepra; dieta; epilèpsiamalaltialcsh:Philology. Linguisticslepralcsh:P1-1091:CIENCIAS DE LAS ARTES Y LAS LETRAS [UNESCO]lcsh:Phospitals
researchProduct