x
uploads/prion.jpg

prion n.【生物化學】朊病毒,感染性蛋白質。

prior

Such examples are given as the blood filtering principal of hemoglobin , catalysis of enzymes , immune recoglization , prion , glycoprotein and the relationship of structure and function of membrane protein , et al , as well as the applications to medicine 在簡要介紹結構生物學的研究方法的基礎上,主要從分子水平闡述蛋白質和核酸的結構原理、相互作用、結構與功能的關系,通過具體實例闡述血紅蛋白的輸氧機制、酶的催化機制、免疫分子識別、朊病毒、糖蛋白、生物膜的結構功能關系等,以及結構生物學在醫學上的應用。

The propagation mechanism of the prion diseases is widely accepted that prpsc transforms prpc into the pathological form and more and more prpsc will gradually be produced and forms larger fibrillar aggregates . when a critical level of prpsc has accumulated , the neurons die , resulting in large vacuoles . the affected brain regions become sponge - like in appearance , hence the name spongiform encephalopathies to describe all prion diseases 目前普遍接受的朊蛋白疾病發生機制為prpsc轉化prpc為致病形式,致使越來越多的致病蛋白形成并堆積成越來越大的纖維狀聚集物,當致病蛋白聚集到一定程度,造成神經元死亡而使受累腦區呈現海綿樣空洞,這也就是為什么用海綿樣腦病來描述所有的骯蛋白疾病。

There exist two prion protein forms , the pathological form , prpsc ( sc stands for scrapie ) , and normal cellular form , prpc ( c stands for cellular ) . these two proteins have the same amino acid se quence , the only difference lies in conformation 研究得知朊蛋白有兩種形式,一種為致病形式( prpsc , sc是瘙癢癥的縮寫) ,另一種為組織內正常存在的細胞形式( prpc , c是細胞型的縮寫) ,這兩種蛋白在一級結構的氨基酸序列相同,僅構象有不同。

Key among these tools are highly sensitive tests , some available and some under development , that can detect prions even in asymptomatic individuals ; currently bse is diagnosed only after an animal has died naturally or been slaughtered 其中幾個關鍵工具就是高靈敏度的檢測方法(有的已經上市,有的則還在研發當中) ,它們可以從沒有癥狀的牛只身上偵測到普恩蛋白的存在;而目前bse的診斷只有在動物自然死亡或屠宰之后才能確認。

The facts that cellular prion protein is extensively distributed among tissues , its high similarity in different species and its indispensability in prion diseases development established its importance in the study of prion diseases 細胞型骯蛋白( prpc )在組織內分介廣泛、在物種問高度保守并且在骯蛋白疾病的發生中不可或缺,這些特點決定了它在骯蛋白疾病研究中的重要地位。

If a drug could be made that inhibits this fragmentation process , it could substantially slow the spread of prions , which cause mad cow disease and scrapie in animals and , in rare cases , creutzfeldt - jacob disease and kuru in humans 藥物可阻滯這些蛋白裂解片段過程,從而持久的減慢朊病毒傳播,包括瘋牛病和綿羊瘋癢病和一些人類罕見疾病克-雅二氏病和庫魯病等疾病的傳播。

A single protein plays a major role in deadly prion diseases by smashing up clusters of these infectious proteins , creating the “ seeds “ that allow fatal brain illnesses to quickly spread , new brown uniersity research shows 布朗大學最新研究顯示,有一種蛋白可能是朊毒病中關鍵蛋白,裂解感染性蛋白生成“種子”片段,然后侵襲腦組織致宿主迅速死亡。

The prototypic prion disease found is scrapie . the epidemic of bse in england and the newly found vcjd in human attracted more attention to prion diseases and the disease - causing proteins 羊瘙癢癥是最早發現的朊蛋白病,而瘋牛病在英國的流行以及新近發現的新變種的人類雅各布氏病引起研究者們對朊蛋白疾病及其致病蛋白的更大關注。

The evidence is accumulating that prion diseases are due to proteins which change their conformation irreversibly during an association reaction , perhaps by mechanisms related to amyloid accumulation 越來越多的證據指出蛋白感染素疾病(如狂牛病)是因為蛋白質結合過程中其構型產生不可逆的改變,可能是由類淀粉體堆積相關的機制造成。

Its breakout in europe caused a great loss and society turbulence and there have been intensive researches on prion about its origin and effective therapy in life science in recent years 以瘋牛病為例,近年來它在歐洲的大爆發引起巨大的財產損失和社會動蕩,因此研究瘋牛病的致病機理及有效治療方法是生命科學領域的熱點課題之一。

As researchers learn more about the challenges posed by prions ? which can incubate without symptoms for years , even decades ? they uncover strategies that could better forestall epidemics 普恩蛋白疾病的潛伏期可長達數年甚至數十年之久,而在研究人員更加了解普恩蛋白帶來的挑戰后,他們找到了更好的方法,可以用來防?大流行。

The history , situation , clinical symptom , detection of bse , hypothesis of prion and protein , mechanism of bse were reviewed . and the possibility of curing bse was elucidated 摘要論述了瘋牛病的發病歷史與現狀、瘋牛病的臨床癥狀與檢測、朊蛋白與蛋白假說和瘋牛病的致病機理等。此外,還討論了治愈瘋牛病的可能性。

It was found that prion which is made up of proteins exclusively is the main cause and it has two forms , i . e , normal form prpc and abnormal one prpsc 目前已知瘋牛病的致病因子是完全由蛋白質組成的朊病毒( prion ) ,它存在“正常”和“致病”兩種狀態,分別被稱為prp ~ c和prp ~ sc 。

By carefully measuring the rate at which proteasomes laboured , she found a clear correlation : as the clumps of prions in the sample got bigger , the proteasomes slowed down 通過測試這些水解酶的工作速率,她發現了一個清晰的關系:當標本中的朊病毒團變大時水解酶的工作便慢了下來。

They occur when normal prion proteins become knotted and resistant to being broken down , and cause rapid brain degeneration that is ineitably fatal 當正常的朊病病毒多節化并具有了抗破壞能力的時候,這些疾病開始出現;接著導致不可避免的致命的迅速腦部惡化。

Preious work has shown that mice with their prion gene knocked out remain healthy , and when inoculated with scrapie are protected against the disease1 先前的工作已經告訴我們,被敲除普粒子基因的老鼠仍然健康,并且當嫁接羊瘙癢病的時候它們能夠抵御疾病。

Researchers have also made some headway in treating a human prion disorder called creutzfeldt - jakob disease ( cjd ) , which today is uniformly fatal 另外,研究人員也在治療庫賈氏癥( cjd )方面取得某些進展,人類的這種普恩蛋白疾病在今天還是無藥可治的絕癥。

The resilience of misfolded prions appears to be a key reason why chronic wasting disease has persisted and spread from its presumed starting point near fort collins , colo 結構異常的普恩蛋白適應力強,可能是慢性消耗病存續并擴散的關鍵因素。

Although these tests show that prions can force the waste disposal system to malfunction , they did not identify which part of the process went wrong 盡管這些實驗證明了朊病毒可以使廢物處理系統故障,但它們并沒有分辨出究竟是哪個環節出了錯。