Hatcher Kristen, Zheng Jian, Chen Shu G
Department of Pathology and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH 44106, USA.
J Alzheimers Dis. 2009;16(2):421-31. doi: 10.3233/JAD-2009-0980.
Prion diseases are a group of fatal neurodegenerative disorders characterized by the accumulation of a misfolded form (PrP(Sc)) of the cellular prion protein (PrP(C)) in the brains of affected individuals. The conversion of PrP(C) to PrP(Sc) is thought to involve a change in protein conformation from a normal, primarily alpha-helical structure into a beta-sheet conformer. Few proteins have been identified that differentially interact with the two forms of PrP. It has been reported that plasminogen binds to PrP(Sc) from a variety of prion phenotypes. We have examined potential motifs within the kringle region that may be responsible for binding to PrP. We synthesized 12-15-mer peptides that contain small, repetitive stretches of amino acid residues found within the kringle domains of plasminogen. These synthetic peptides were found to capture PrP(Sc) from the brain homogenates of bovine spongiform encephalopathy affected cattle, chronic wasting disease affected elk, experimental scrapie of hamsters and that of subjects affected by Creutzfeldt-Jakob disease, without binding to PrP(C) in unaffected controls. Therefore, we have identified critical peptide motifs that may be important for protein-protein interactions in prion disease pathogenesis. The ability of these synthetic peptides to bind preferentially to PrP(Sc) suggests a potential application in the diagnosis of prion diseases.
朊病毒疾病是一组致命的神经退行性疾病,其特征是在受影响个体的大脑中,细胞朊蛋白(PrP(C))的错误折叠形式(PrP(Sc))积累。PrP(C)向PrP(Sc)的转变被认为涉及蛋白质构象从正常的主要为α-螺旋结构转变为β-折叠构象。很少有蛋白质被鉴定出与PrP的两种形式有差异相互作用。据报道,纤溶酶原可与多种朊病毒表型的PrP(Sc)结合。我们研究了kringle区域内可能负责与PrP结合的潜在基序。我们合成了12 - 15聚体肽,这些肽包含纤溶酶原kringle结构域内发现的小的、重复的氨基酸残基片段。这些合成肽被发现可从感染牛海绵状脑病的牛、感染慢性消耗病的麋鹿、仓鼠实验性瘙痒病以及克雅氏病患者的脑匀浆中捕获PrP(Sc),而在未受影响的对照中不与PrP(C)结合。因此,我们确定了关键的肽基序,这些基序在朊病毒疾病发病机制中的蛋白质 - 蛋白质相互作用中可能很重要。这些合成肽优先结合PrP(Sc)的能力表明其在朊病毒疾病诊断中的潜在应用。