Biomaterial Database

Differences in the membrane interaction of scrapie amyloid precursor proteins in normal and scrapie- or Creutzfeldt-Jakob disease-infected brains. 1991

J Safar, and M Ceroni, and D C Gajdusek, and C J Gibbs

Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.

The membrane interaction and hydrophobicity of the normal (PrPC) and infectious isoform (PrPSc/CJD) of scrapie and Creutzfeldt-Jakob disease amyloid precursor proteins was studied. The normal isoform of hamster and human scrapie amyloid precursor protein was found on the microsomal/synaptosomal membranes anchored solely by the C-terminal glycolipid. Glycolipid cleavage resulted in dissociation from the membranes and change of behavior from a highly hydrophobic to a hydrophilic protein, susceptible to proteases. In contrast, the PrPSc/CJD isoform was resistant to release by glycolipid-cleaving enzymes. A part of PrPSc/CJD was released from the membranes after prolonged trypsin treatment, yielding a further protease-resistant product of 27-30 kDa. The results demonstrate the proteolytic resistance of the membrane-bound PrPSc/CJD isoform and also indicate the presence of a different, apparently disease-induced mechanism of membrane interaction in the scrapie- and CJD-infected microsomal and synaptosomal membranes.

UI	MeSH Term	Description	Entries
D007562	Creutzfeldt-Jakob Syndrome	A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ATAXIA; APHASIA, visual loss, weakness, muscle atrophy, MYOCLONUS, progressive dementia, and death within one year of disease onset. A familial form exhibiting autosomal dominant inheritance and a new variant CJD (potentially associated with ENCEPHALOPATHY, BOVINE SPONGIFORM) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of PRIONS. (From N Engl J Med, 1998 Dec 31;339(27))	New Variant Creutzfeldt-Jakob Disease,Spongiform Encephalopathy, Subacute,CJD (Creutzfeldt-Jakob Disease),Creutzfeldt Jacob Disease,Creutzfeldt-Jakob Disease,Creutzfeldt-Jakob Disease, Familial,Creutzfeldt-Jakob Disease, New Variant,Creutzfeldt-Jakob Disease, Variant,Familial Creutzfeldt-Jakob Disease,Jakob-Creutzfeldt Disease,Jakob-Creutzfeldt Syndrome,V-CJD (Variant-Creutzfeldt-Jakob Disease),Variant Creutzfeldt-Jakob Disease,CJD (Creutzfeldt Jakob Disease),Creutzfeldt Jakob Disease,Creutzfeldt Jakob Disease, Familial,Creutzfeldt Jakob Disease, New Variant,Creutzfeldt Jakob Disease, Variant,Creutzfeldt Jakob Syndrome,Creutzfeldt-Jakob Diseases, Familial,Disease, Creutzfeldt Jacob,Disease, Creutzfeldt-Jakob,Disease, Familial Creutzfeldt-Jakob,Disease, Jakob-Creutzfeldt,Encephalopathies, Subacute Spongiform,Encephalopathy, Subacute Spongiform,Familial Creutzfeldt Jakob Disease,Familial Creutzfeldt-Jakob Diseases,Jacob Disease, Creutzfeldt,Jakob Creutzfeldt Disease,Jakob Creutzfeldt Syndrome,New Variant Creutzfeldt Jakob Disease,Spongiform Encephalopathies, Subacute,Subacute Spongiform Encephalopathies,Subacute Spongiform Encephalopathy,Syndrome, Creutzfeldt-Jakob,Syndrome, Jakob-Creutzfeldt,V CJD (Variant Creutzfeldt Jakob Disease),Variant Creutzfeldt Jakob Disease
D008297	Male		Males
D008647	Mesocricetus	A genus in the order Rodentia and family Cricetidae. One species, Mesocricetus auratus or golden hamster is widely used in biomedical research.	Hamsters, Golden,Hamsters, Golden Syrian,Hamsters, Syrian,Mesocricetus auratus,Syrian Golden Hamster,Syrian Hamster,Golden Hamster,Golden Hamster, Syrian,Golden Hamsters,Golden Syrian Hamsters,Hamster, Golden,Hamster, Syrian,Hamster, Syrian Golden,Syrian Hamsters
D010450	Endopeptidases	A subclass of PEPTIDE HYDROLASES that catalyze the internal cleavage of PEPTIDES or PROTEINS.	Endopeptidase,Peptide Peptidohydrolases
D011498	Protein Precursors		Precursors, Protein
D012016	Reference Values	The range or frequency distribution of a measurement in a population (of organisms, organs or things) that has not been selected for the presence of disease or abnormality.	Normal Range,Normal Values,Reference Ranges,Normal Ranges,Normal Value,Range, Normal,Range, Reference,Ranges, Normal,Ranges, Reference,Reference Range,Reference Value,Value, Normal,Value, Reference,Values, Normal,Values, Reference
D001921	Brain	The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.	Encephalon
D005260	Female		Females
D006017	Glycolipids	Any compound containing one or more monosaccharide residues bound by a glycosidic linkage to a hydrophobic moiety such as an acylglycerol (see GLYCERIDES), a sphingoid, a ceramide (CERAMIDES) (N-acylsphingoid) or a prenyl phosphate. (From IUPAC's webpage)	Glycolipid
D006224	Cricetinae	A subfamily in the family MURIDAE, comprising the hamsters. Four of the more common genera are Cricetus, CRICETULUS; MESOCRICETUS; and PHODOPUS.	Cricetus,Hamsters,Hamster