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中文摘要：
朊病毒病是一类人畜共患的致死性神经退行性疾病，尚无有效的预防和治疗手段。目前研究表明，正常PrP蛋白（PrPC）和异常的PrP蛋白（PrPSc）在体内存在着不同的剪切形式。为了更好的探明朊病毒病的致病机制，本课题在我们现有的Prion疾病动物模型及错误蛋白扩增技术（PMCA）的基础上，筛选几种可能参与PrPC和PrPSc剪切的内肽酶，进行PrP蛋白的体外剪切实验，找到体内真正参与PrP蛋白剪切的内肽酶，为阐明PrP蛋白的剪切机制奠定基础。同时，在细胞水平对不同剪切片段进行毒性实验，以了解这些剪切片段对神经细胞的毒性作用。而且，通过有毒性的C2片段是否可以进一步剪切产生C1片段的研究，为预防和治疗Prion疾病提供新的思路。
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期刊论文
Mouse-adapted scrapie strains 139A and ME7 overcome species barrier to induce experimental scrapie i
Immune responses in wild-type mice against prion proteins induced using a DNA prime-protein boost st
Raised levels of tau protein in CSF of Chinese patients with probable diagnostic Creutzfeldt-Jakob d
The octarepeats of hamster PrP (PrP51-91) enhance the formation microtubule and antagonize Cu2+-indu
PrPSc of scrapie 263K propagates efficiently in spleen and muscle tissues with protein misfolding cy
Molecular interaction of TPPP with PrP antagonized the CytoPrP-induced disruption of microtubule str
Familial CJD associated PrP mutants within transmembrane region induced retention of Ctm-PrP in ER a
A novel PrP partner HS-1 associated protein X-1 (HAX-1) protected the cultured cells against the cha
The epidemiological, clinical and laboratory features of sporadic Creutzfeldt-Jakob disease patients
The diversities of PrPSc distributions and pathologic changes in various brain regions from a Chines
Comparative peptidome analyses of the profiles of the peptides ranging from 1 to 10 KD in CSF sample
Knocking-down of the levels of prion protein by RNA interference (RNAi) weakened the protective acti
Remarkable reduction of MAP2 in the brains of scrapie-infected rodents and human prion disease possi
Transient expressions of doppel and its structural analog prionΔ32-121 inSH-SY5Y cells caused cytoto
Molecular interaction of alpha-synuclein with tubulin influences onthe polymerization of microtubule
Changes of tau profiles in brains of the hamsters infected with scrapie strains263K or 139A possibly
Manganese induced changes of the biochemical characteristics of therecombinant wild-type and mutated
Cytosolic prion protein induces apoptosis inhuman neuronal cell SH-SY5Y via mitochondrial disruption
The propagation of hamster-adapted scrapie PrPSc can be enhanced by reduced pyridine nucleotide in v
Human prion protein mutants with deleted and inserted octarepeats undergo in different pathways to t
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