Detection and Characterization of Blood-borne Prions

目前还没有实用的非侵入性死前检测方法来检测梅毒、人类或任何一种普恩病毒疾病。血- 无症状人类变异克雅氏病(VCJD)感染的普恩病毒传播 人类已经发生(12，14-16)。慢性消耗性疾病(CWD)是一种急诊、快速传播的疾病。 鹿类(27、28、37-40)具有不确定的人畜共患潜力(3-8)。据估计，每36个美国人中就有一个猎鹿和/或 麋鹿，导致美国每年估计有7,000-15,000头感染CWD的鹿肉被消费(不断增加 20%/年)(2)。由于狩猎是一个每年约260亿美元的行业(1)，CWD代表着巨大的潜力 健康和经济威胁。VCJD和Cervid CWD之间的共同点，包括 血源性感染性(34，47)，显著。对人类的宰前检测和监测，以及 在动物身上，Pron病已经被低浓度的Prion和丰富的 血液和其他可获得的生物样本中的化验抑制物。我们已经开发出简单、快速、 特异性和高灵敏度的体外检测血液传播的普恩病毒(9-11)。在这场竞争性更新中，我们将 对连续采集的丙种球蛋白血症的时间分布和特定血细胞表型进行量化 人和宫颈的血样。 这些研究结果的翻译影响将： A)提高人体输血安全， B)提供更好的手段来评估和监测发展中的普恩疗法， C)在很大程度上消除人类食用鹿蛋白的风险， D)更好地了解CWD的流行情况和对鹿类狩猎的后果 农业产业，以及 E)阐明我们对原蛋白血症在疾病发病机制中的理解。 因此，这项研究将对人类和动物的健康产生直接影响。

No practical noninvasive antemortem test exists to detect cervid, human, or any, prion disease. Blood- borne transmission of prions from asymptomatic human variant Creutzfeldt Jakob Disease (vCJD)-infected humans has occurred(12,14-16). Chronic wasting disease (CWD) is an emergent rapidly spreading disease in cervids(27,28,37-40) with uncertain zoonotic potential(3-8). An estimated one in 36 Americans hunt deer and/or elk, resulting in an estimated 7,000-15,000 CWD-infected cervids consumed annually in the USA (increasing 20%/yr)(2). As hunting is a roughly $26 billion (per annum) industry(1), CWD represents an enormous potential health and economic threat. The commonalities among vCJD and cervid CWD, including the presence of hematogenous infectivity(34,47), are remarkable. Antemortem detection and surveillance of human, and animal, prion disease has been hindered by the combination of low prion concentrations and abundant assay inhibitors in blood and other accessible biological samples. We have developed simple, rapid, specific and highly sensitive in vitro assays to detect blood-borne prions(9-11). In this competing renewal we will quantitate the temporal distribution and specific blood cell phenotypes critical to prionemia in serially-collected human and cervid blood samples. The translational impact of the results of these studies will: a) improve human blood transfusion safety, b) provide a better means to assess and monitor developing prion therapeutics, c) largely eliminate the risks of human consumption of cervid prions, d) provide greater understanding of the prevalence and consequences of CWD to the cervid hunting and agriculture industries, and e) elucidate our understanding of prionemia in disease pathogenesis. Thereby, this research will have immediate impacts in both human and animal health.