Analyses of Gerstmann-Straussler syndrome with 102Leu219Lys using monoclonal antibodies that specifically detect human prion protein with 219Glu
Analyses of Gerstmann-Straussler syndrome with 102Leu219Lys using monoclonal antibodies that specifically detect human prion protein with 219Glu
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DOI：
10.1016/s0304-3940(00)01232-5
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发表时间：
2000-07-21
影响因子：
2.5
通讯作者：
Kitamoto, T
中科院分区：
文献类型：
--
作者：
Muramoto, T;Tanaka, T;Kitamoto, T
开发了两种特异性检测人朊病毒蛋白 (PrP) 的单克隆抗体。使用谷胱甘肽-S-转移酶和 PrP 肽的融合蛋白将两种抗体的表位定位到包含多态性 219 残基的 C 末端区域。这些抗体可识别带有 219Glu 的人 PrP，但不识别带有 219Lys 的人 PrP。利用抗体的独特特性来确定沉积在格斯特曼-施特劳斯勒综合征 (GSS) 患者大脑中的异常 PrP 的等位基因起源，其中 102Leu/219Lys 由相同等位基因编码。异常 PrP 完全是突变等位基因来源，表明 219Lys 可能允许 GSS 中异常 PrP 的形成。该抗体可能有助于探讨219Glu/Lys多态性与人类朊病毒疾病发病机制的关系。 (C) 2000 Elsevier Science Ireland Ltd. 保留所有权利。
Two monoclonal antibodies that specifically detect human prion protein (PrP) were developed. The epitope of both antibodies was mapped using fusion proteins of glutathione-S-transferase and PrP peptides to the C-terminal region encompassing the polymorphic 219 residue. The antibodies recognized human PrP with 219Glu but not that with 219Lys. The unique property of the antibodies was utilized to determine the allelic origin of abnormal PrP deposited in the brain of a patient with Gerstmann-Straussler syndrome (GSS) with 102Leu/219Lys encoded by the same allele. Abnormal PrP was exclusively of mutant allelic origin, suggesting that 219Lys may be permissive to the formation of abnormal PrP in GSS. The antibodies may help to explore the relationship of 219Glu/Lys polymorphism to the pathogenesis of human prion diseases. (C) 2000 Elsevier Science Ireland Ltd. All rights reserved.