PMID- 7904883 OWN - NLM STAT- MEDLINE DCOM- 19940302 LR - 20191023 IS - 1350-7540 (Print) IS - 1350-7540 (Linking) VI - 6 IP - 6 DP - 1993 Dec TI - Alzheimer's disease and Creutzfeldt-Jakob disease: overlap of pathogenic mechanisms. PG - 872-81 AB - This article compares beta-amyloid precursor protein (beta-APP) disorders exemplified by Alzheimer's disease (AD), with prion protein (PrP) disorders, exemplified by Creutzfeldt-Jakob disease (CJD) in humans and scrapie in animals. Although there are obvious differences in the etiology and pathogenesis of both sets of disorders, a remarkable number of similarities exist. Both sets of disorders are characterized clinically by age-related sporadic and familial diseases. In both, an abnormal form of a neuronal membrane protein appears to play a key role in the pathogenesis: beta-A4 peptide in AD and PrPCJD in CJD. Both beta-A4 and PrPCJD are amyloidogenic. Neuritic plaques characteristic of AD were once thought to be exclusively associated with beta-A4 amyloid; however, some pedigrees with familial prion disease produced neuritic plaques with PrP amyloid cores. Finally, beta-APP accumulation in skeletal muscle has been implicated in the age-related muscle disorder, inclusion body myositis. A similar myopathy has recently been discovered in transgenic mice expressing high levels of normal PrP. These similarities suggest that what is learned about one set of disorders may be applicable to the other. FAU - DeArmond, S J AU - DeArmond SJ AD - Department of Pathology, University of California, San Francisco 94143-0506. LA - eng GR - AG02132/AG/NIA NIH HHS/United States GR - NS14069/NS/NINDS NIH HHS/United States GR - NS22786/NS/NINDS NIH HHS/United States GR - etc. PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, P.H.S. PT - Review PL - England TA - Curr Opin Neurol JT - Current opinion in neurology JID - 9319162 RN - 0 (Amyloid beta-Protein Precursor) RN - 0 (PrPSc Proteins) RN - 0 (Prions) SB - IM MH - Aged MH - Alzheimer Disease/*etiology/genetics/pathology MH - Amyloid beta-Protein Precursor/*analysis/genetics MH - Animals MH - Brain/pathology MH - Creutzfeldt-Jakob Syndrome/*etiology/genetics/pathology MH - Disease Models, Animal MH - Humans MH - Mutation/genetics MH - PrPSc Proteins MH - Prions/*analysis/genetics RF - 76 EDAT- 1993/12/01 00:00 MHDA- 1993/12/01 00:01 CRDT- 1993/12/01 00:00 PHST- 1993/12/01 00:00 [pubmed] PHST- 1993/12/01 00:01 [medline] PHST- 1993/12/01 00:00 [entrez] AID - 10.1097/00019052-199312000-00008 [doi] PST - ppublish SO - Curr Opin Neurol. 1993 Dec;6(6):872-81. doi: 10.1097/00019052-199312000-00008.