0000000000455627

AUTHOR

Hans A. Kretzschmar

showing 3 related works from this author

Impact of vCJD on blood supply.

2007

Variant Creutzfeldt-Jakob disease (vCJD) is an at present inevitably lethal neurodegenerative disease which can only be diagnosed definitely post mortem. The majority of the approximately 200 victims to date have resided in the UK where most contaminated beef materials entered the food chain. Three cases in the UK demonstrated that vCJD can be transmitted by blood transfusion. Since BSE and vCJD have spread to several countries outside the UK, it appears advisable that specific risk assessments be carried out in different countries and geographic areas. This review explains the approach adopted by Germany in assessing the risk and considering precautionary measures. A fundamental premise is…

medicine.medical_specialtyBlood transfusionBovine spongiform encephalopathymedicine.medical_treatmentSpecific riskBioengineeringBlood Component TransfusionBlood DonorsDiseaseApplied Microbiology and BiotechnologyCreutzfeldt-Jakob SyndromeBlood Component TransfusionEnvironmental healthGermanymental disordersmedicineBlood-Borne PathogensAnimalsHumansLeukapheresisPharmacologyGeneral Immunology and Microbiologybusiness.industryIncidencePatient SelectionTransfusion ReactionGeneral MedicineRuminantsmedicine.diseasenervous system diseasesSurgeryEncephalopathy Bovine SpongiformPopulation dataBlood supplyCattleRisk assessmentbusinessBiotechnologyBiologicals : journal of the International Association of Biological Standardization
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Influence of ADAM10 on prion protein processing and scrapie infectiosity in vivo.

2009

Abstract Both the cellular prion protein (PrPc) and the amyloid precursor protein (APP) are physiologically subjected to complex proteolytic processing events. While for APP the proteinases involved – alpha-, beta- and gamma-secretase – have been identified in vitro and in vivo, the cleavage of PrPc by now has been linked only to the shedding activity of the metalloproteinase ADAM10 and/or ADAM17 in cell culture. Here we show that neuronal overexpression of the alpha-secretase ADAM10 in mice reduces all PrPc species detected in the brain instead of leading to enhanced amounts of specific cleavage products of PrPc. Additionally, the incubation time of mice after scrapie infection is signific…

Prionsanimal diseasesADAM10Molecular Sequence DataPrion diseaseScrapieMice Transgeniclcsh:RC321-571ADAM10 ProteinMiceIn vivomental disordersNeurotoxicitymedicineAmyloid precursor proteinAnimalsHumansGliosisAmino Acid Sequencealpha-Secretaselcsh:Neurosciences. Biological psychiatry. NeuropsychiatrySheddingMetalloproteinasebiologyChemistryBrainMembrane ProteinsMolecular biologyIn vitronervous system diseasesMice Inbred C57BLADAM ProteinsNeurologyAlpha secretaseGliosisbiology.proteinCattlemedicine.symptomAmyloid Precursor Protein SecretasesProtein Processing Post-TranslationalScrapieNeurobiology of disease
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Immunohistochemistry of primary central nervous system malignant rhabdoid tumors: report of five cases and review of the literature

1996

Malignant rhabdoid tumors (MRT) are characterized by a typical light microscopic morphology with uniformly round tumor cells, vacuolated cytoplasm with occasional round, hyaline intracytoplasmic, periodic acid-Schiff-positive inclusions, vesicular nuclei with prominent nucleoli and positive immunoreactivity for vimentin. The histogenesis of MRT is controversial. Five cases of primary central nervous system (CNS) rhabdoid tumors in children are presented. Immunohistochemical, light and electron microscopic features are compared with primary CNS malignant rhabdoid tumors reported in the literature. Expression of various neurofilaments in our cases of primary CNS rhabdoid tumors was prominent …

Central Nervous SystemMalePathologymedicine.medical_specialtyNeurofilamentCentral nervous systemVimentinHistogenesisBiologyPathology and Forensic MedicineCentral nervous system disease03 medical and health sciencesCellular and Molecular Neuroscience0302 clinical medicinemedicineHumansRhabdoid TumorHyalineBrain NeoplasmsRhabdoid tumorsInfantmedicine.diseaseImmunohistochemistry3. Good healthMicroscopy Electronmedicine.anatomical_structureChild Preschool030220 oncology & carcinogenesisbiology.proteinImmunohistochemistryFemaleNeurology (clinical)030217 neurology & neurosurgeryActa Neuropathologica
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