Scrapie agent proteins do not accumulate in grey tremor mice

• Published in: Journal of general virology Vol. 69; no. 4; pp. 961 - 966
• Main Authors: Bendheim, P.E, Marmorstein, A.D, Potempska, A, Bolton, D.C
• Format: Journal Article
• Language: English
• Published: Reading Soc General Microbiol 01-04-1988; Society for General Microbiology
• Subjects: Animals; Biological and medical sciences; Brain Diseases - genetics; Brain Diseases - microbiology; Fundamental and applied biological sciences. Psychology; Mice; Mice, Inbred C57BL - microbiology; Mice, Neurologic Mutants - microbiology; Microbiology; Prions - analysis; Prions - pathogenicity; proteins; PrPSc proteins; recessive genes; Replicative cycle, interference, host-virus relations, pathogenicity, miscellaneous strains; Scrapie - microbiology; strain differences; Viral Proteins - analysis; Virology; Virus; Proteins; Vertebrata; Mammalia; Mouse; Tremor; Animal; Rodentia; Mutation; Scrapie agent; Homozygozity; Strain
• ISSN: 0022-1317; 1465-2099
• DOI: 10.1099/0022-1317-69-4-961

1 Department of Pathological Neurobiology
and 2 Department of Molecular Biology, New York State Institute for Basic Research in Developmental Disabilities, 1050 Forest Hill Road, Staten Island, New York 10314, U.S.A.
The grey tremor mouse is an autosomal recessive mutant characterized by a phenotype of unusual pigmentation, neurological abnormalities and early death. These mice have a spongiform encephalopathy similar to scrapie and Creutzfeldt-Jakob disease. Although the disease is clearly heritable, the grey tremor mouse spongiform pathology has also been transmitted by inoculation of genetically normal mice with diseased brain homogenates. The possibility that a scrapie-like agent is involved has been proposed. We examined brain homogenates from grey tremor mice, scrapie-affected mice and normal mice for the presence of the mouse scrapie agent protein (MoSp33–37) and its normal cellular homologue. All untreated homogenates contained one or both isoforms of this protein as detected on immunoblots. Grey tremor mouse brain homogenates, when protease-treated, showed no evidence of MoSp33–37. A purification method for MoSp33–37 concentrated it in samples from scrapie-affected mice, but this protein was not detected in grey tremor or normal mice. These results suggest that it is unlikely that the scrapie agent is involved in grey tremor disease.
Keywords: scrapie agent protein, grey tremor mice, transmissible spongiform encephalopathy
Received 12 October 1987;
accepted 18 December 1987.