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© 2020 WILEY-VCH Verlag GmbH Co. KGaA, Weinheim.BACKGROUND Indigenous Australians are of increased risk of developing dementia - Alzheimer's disease and mixed dementia diagnoses are the most common. Whilst prion diseases have been reported in Indigenous peoples of Papua New Guinea and the United States of America, the occurrence and phenotype of prion disease in Indigenous Australians is hitherto unreported. AIM Report the incidence rate and clinical phenotype of Creutzfeldt-Jakob disease (CJD) in Indigenous Australians. METHOD Calcu