Expression of the cellular prion protein (PrPC) is crucial for the development of prion diseases. Amino acid changes in PrPC or a reduced amount of PrPC may modulate disease resistance. The relative abundance of C1, a natural α-cleavage fragment of PrPC, was previously found to be associated with a resistant PRNP genotype in sheep. Goats are another small ruminant where classical scrapie susceptibility is under strong genetic control. In this study, we assessed PrPC in goats for the existence of similar associations between PrPC fragments and genotype. Brain tissue homogenates from scrapie-free goats with wild type PRNP or polymorphisms (I142M, H143R, N146S, or Q222K) were deglycosylated prior to immunoblot for assessment of the relative abundance of the C1 fragment of PrPC. The presence of K222 or S146 alleles demonstrated significantly different relative levels of C1 compared to that observed in wild type goats, which suggests that the genotype association with C1 is neither unique to sheep nor exclusive to the ovine Q171R dimorphism.

中文翻译：

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山羊 PRNP 多态性 N146S 和 Q222K 与 PrPC 的蛋白水解裂解有关

细胞朊病毒蛋白 (PrPC) 的表达对于朊病毒疾病的发展至关重要。PrPC 中的氨基酸变化或 PrPC 的量减少可能会调节抗病性。C1 是 PrPC 的天然 α 切割片段，其相对丰度先前被发现与绵羊的抗性 PRNP 基因型相关。山羊是另一种小型反刍动物，其中经典的痒病易感性受到强烈的遗传控制。在这项研究中，我们评估了山羊中的 PrPC 是否存在 PrPC 片段和基因型之间的类似关联。来自具有野生型 PRNP 或多态性（I142M、H143R、N146S 或 Q222K）的无痒病山羊的脑组织匀浆在免疫印迹之前被去糖基化，以评估 PrPC 的 C1 片段的相对丰度。