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An emerging concept of prion infections as a form of transmissible cerebral amyloidosis.
Prion ( IF 1.9 ) Pub Date : 2007-10-01 , DOI: 10.4161/pri.1.4.5816
Omar Lupi 1 , Marcius Achiame Peryassu
Affiliation  

Proteins are a major constituent of cells with specific biological functions. Besides the primary structure that is simply the sequence of amino acids that comprise a protein, the secondary structure represents the first step of folding defining its general conformation. The biological functions of proteins are directly dependent on the acquisition of their conformation. The same protein can have different stable states, which may participate with different functions in the cell. The amyloid diseases comprise Alzheimer's and Parkinson's diseases, type II diabetes mellitus and systemic amyloidosis. Amyloid fibers are insoluble, resistant to proteolysis and show an extremely high content of beta-sheet, in a very similar structure to the one observed among prion rods, associated to the transmissible spongiform encephalopathies. All these diseases are "infectious" in the sense that misfolded beta-sheeted conformers formed in a nucleation process in which preformed metastable oligomer acts as a seed to convert a normal isoform into an abnormal protein with a misfolded conformation. Only prion infections have a proven infectivity in a microbiological sense; some recent observations, however, detected the transmissibility of systemic amyloidosis by a prion-like mechanism among mice. Prions diseases and amyloidosis present many similar aspects of the so-called conformational diseases; according to this interpretation the prion infections could be considered as a form of transmissible cerebral amyloidosis.

中文翻译:

朊病毒感染作为一种传染性脑淀粉样变性的新概念。

蛋白质是具有特定生物学功能的细胞的主要成分。除了构成蛋白质的简单氨基酸序列的一级结构外,二级结构代表折叠定义其一般构象的第一步。蛋白质的生物学功能直接取决于其构象的获得。同一种蛋白质可以有不同的稳定状态,在细胞中可能参与不同的功能。淀粉样疾病包括阿尔茨海默病和帕金森病、II型糖尿病和系统性淀粉样变性。淀粉样纤维是不溶性的,对蛋白水解有抵抗力,并显示出极高含量的 β-折叠,其结构与在朊病毒杆中观察到的结构非常相似,与传染性海绵状脑病相关。所有这些疾病都是“传染性的”,因为在成核过程中形成了错误折叠的 β-折叠构象异构体,在该过程中,预先形成的亚稳寡聚体充当种子,将正常同工型转化为具有错误折叠构象的异常蛋白质。只有朊病毒感染才具有微生物学意义上的传染性;然而,最近的一些观察发现,系统性淀粉样变性通过类似朊病毒的机制在小鼠中传播。朊病毒病和淀粉样变性与所谓的构象病有许多相似之处;根据这种解释,朊病毒感染可被视为一种传染性脑淀粉样变。从某种意义上说,错误折叠的β-折叠构象异构体在成核过程中形成,在该过程中,预先形成的亚稳寡聚体充当种子,将正常同工型转化为具有错误折叠构象的异常蛋白质。只有朊病毒感染才具有微生物学意义上的传染性;然而,最近的一些观察发现,系统性淀粉样变性通过类似朊病毒的机制在小鼠中传播。朊病毒病和淀粉样变性与所谓的构象病有许多相似之处;根据这种解释,朊病毒感染可被视为一种传染性脑淀粉样变。从某种意义上说,错误折叠的 β-折叠构象异构体在成核过程中形成,在该过程中,预先形成的亚稳寡聚体充当种子,将正常同工型转化为具有错误折叠构象的异常蛋白质。只有朊病毒感染才具有微生物学意义上的传染性;然而,最近的一些观察发现,系统性淀粉样变性通过类似朊病毒的机制在小鼠中传播。朊病毒病和淀粉样变性与所谓的构象病有许多相似之处;根据这种解释,朊病毒感染可被视为一种传染性脑淀粉样变。通过类似朊病毒的机制检测了小鼠系统性淀粉样变性的传播能力。朊病毒病和淀粉样变性与所谓的构象病有许多相似之处;根据这种解释,朊病毒感染可被视为一种传染性脑淀粉样变。通过类似朊病毒的机制检测了小鼠系统性淀粉样变性的传播能力。朊病毒病和淀粉样变性与所谓的构象病有许多相似之处;根据这种解释,朊病毒感染可被视为一种传染性脑淀粉样变。
更新日期:2019-11-01
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