Shadoo (Sprn) and prion disease incubation time in mice.
Shadoo (Sprn) and prion disease incubation time in mice.
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DOI：
10.1007/s00335-009-9194-5
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发表时间：
2009-06
期刊：
影响因子：
2.5
通讯作者：
Collinge, John
中科院分区：
文献类型：
--
作者：
Lloyd, Sarah E.;Grizenkova, Julia;Pota, Hirva;Collinge, John
朊病毒病是哺乳动物物种的可传播的神经退行性疾病，包括羊瘙痒病、牛海绵状脑病（BSE）和变异型克雅氏病（vCJD）。朊病毒蛋白（PrP）在该病中起关键作用，人类和小鼠的编码多态性分别影响疾病易感性和潜伏期。其他基因也被认为是重要的，一个合理的候选者是Sprn，它编码PrP样蛋白Shadoo（Sho）。Sho在成人中枢神经系统中表达，并在体外试验中表现出类似PrP的神经保护活性。为了研究Sprn在朊病毒疾病潜伏期中的作用，我们对一组不同小鼠的开放阅读框（ORF）进行了测序，除了来自野生捕获小鼠的菌株外，几乎没有发现变化。测序的非翻译区揭示多态性，使我们能够进行关联研究的潜伏期在Northport异质股票的小鼠接种钱德勒/RML朊病毒。我们还检测了正常小鼠和朊病毒感染小鼠脑中Sprn mRNA的表达水平，发现与基因型或孵育时间无关。因此，我们的结论是，在这些品系的小鼠中，Sprn在朊病毒疾病的潜伏期中不起主要作用。
Prion diseases are transmissible neurodegenerative disorders of mammalian species and include scrapie, bovine spongiform encephalopathy (BSE), and variant Creutzfeldt-Jakob disease (vCJD). The prion protein (PrP) plays a key role in the disease, with coding polymorphism in both human and mouse influencing disease susceptibility and incubation time, respectively. Other genes are also thought to be important and a plausible candidate is Sprn, which encodes the PrP-like protein Shadoo (Sho). Sho is expressed in the adult central nervous system and exhibits neuroprotective activity reminiscent of PrP in an in vitro assay. To investigate the role of Sprn in prion disease incubation time we sequenced the open reading frame (ORF) in a diverse panel of mice and saw little variation except in strains derived from wild-trapped mice. Sequencing the untranslated regions revealed polymorphisms that allowed us to carry out an association study of incubation period in the Northport heterogeneous stock of mice inoculated with Chandler/RML prions. We also examined the expression level of Sprn mRNA in the brains of normal and prion-infected mice and saw no correlation with either genotype or incubation time. We therefore conclude that Sprn does not play a major role in prion disease incubation time in these strains of mice.
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