NEARLY UBIQUITOUS TISSUE DISTRIBUTION OF THE SCRAPIE AGENT PRECURSOR PROTEIN
NEARLY UBIQUITOUS TISSUE DISTRIBUTION OF THE SCRAPIE AGENT PRECURSOR PROTEIN
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DOI：
10.1212/wnl.42.1.149
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发表时间：
1992-01-01
期刊：
影响因子：
9.9
通讯作者：
BOLTON, DC
中科院分区：
文献类型：
--
作者：
BENDHEIM, PE;BROWN, HR;BOLTON, DC
羊瘙痒病的“修饰的宿主蛋白”模型提出，传播因子由抗降解蛋白Sp33-37组成，并且临床和病理体征是由该蛋白的神经毒性积累引起的。 Sp33-37是正常存在的细胞蛋白Cp 33 -37的异常的淀粉样蛋白生成同种型。 本研究考察了Cp 33 -37在仓鼠体内的组织分布。 在脑内，Cp 33 -37主要集中在海马结构。 免疫组织化学研究定位Cp 33 -37的神经元和周围的neurology在海马，隔，尾状核，丘脑核，背根神经节细胞和大直径背根轴突。 在非神经元仓鼠组织中，在循环白细胞、心脏、骨骼肌、肺、肠道、脾、睾丸、卵巢和一些其他器官中检测到Cp 33 -37。 Cp 33 -37在脑外组织中的存在表明其功能并非脑所独有。 这些结果表明，用于产生Sp33-37、瘙痒症因子和瘙痒症淀粉样蛋白的分子底物存在于多种脑和脑外部位。
The "modified host protein" model of scrapie proposes that the transmissible agent is composed of the degradation-resistant protein, Sp33-37, and that clinical and pathologic signs result from neurotoxic accumulations of this protein. Sp33-37 is an abnormal, amyloidogenic isoform of the normally occurring cellular protein Cp33-37. This study investigated the tissue distribution of Cp33-37 in hamster. In brain, Cp33-37 was most concentrated in the hippocampal formation. Immunohistochemical studies localized Cp33-37 to neurons and surrounding neuropil in hippocampus; septal, caudate, and thalamic nuclei; dorsal root ganglia cells; and large-diameter dorsal root axons. In non-neuronal hamster tissues, Cp33-37 was detected in circulating leukocytes, heart, skeletal muscle, lung, intestinal tract, spleen, testis, ovary, and some other organs. The presence of Cp33-37 in extracerebral tissues indicates that its function is not unique to brain. These results indicate that the molecular substrate for the production of Sp33-37, the scrapie agent, and scrapie amyloid is present in a variety of cerebral and extracerebral sites.