Bendheim P E, Marmorstein A D, Potempska A, Bolton D C
Department of Pathological Neurobiology, New York State Institute for Basic Research in Developmental Disabilities, Staten Island 10314.
J Gen Virol. 1988 Apr;69 ( Pt 4):961-6. doi: 10.1099/0022-1317-69-4-961.
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.
灰色震颤小鼠是一种常染色体隐性突变体，其特征表现为色素沉着异常、神经功能异常以及早期死亡的表型。这些小鼠患有类似于羊瘙痒病和克雅氏病的海绵状脑病。尽管这种疾病具有明显的遗传性，但通过将患病脑匀浆接种给基因正常的小鼠，灰色震颤小鼠的海绵状病理也得以传播。有人提出可能涉及一种类似羊瘙痒病的病原体。我们检测了灰色震颤小鼠、感染羊瘙痒病的小鼠以及正常小鼠的脑匀浆，以确定是否存在小鼠羊瘙痒病病原体蛋白（MoSp33 - 37）及其正常细胞同源物。在免疫印迹检测中，所有未经处理的匀浆都含有该蛋白的一种或两种同工型。经蛋白酶处理后的灰色震颤小鼠脑匀浆未显示出MoSp33 - 37的迹象。一种用于MoSp33 - 37的纯化方法将其浓缩在感染羊瘙痒病小鼠的样本中，但在灰色震颤小鼠或正常小鼠中未检测到这种蛋白。这些结果表明，羊瘙痒病病原体不太可能与灰色震颤病有关。
