Biology and genetics of prions causing neurodegeneration.
Biology and genetics of prions causing neurodegeneration.
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DOI：
10.1146/annurev-genet-110711-155524
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发表时间：
2013
影响因子：
11.1
通讯作者：
Prusiner SB
中科院分区：
文献类型：
--
作者：
Prusiner SB
朊病毒是一种获得可自我繁殖的替代构象的蛋白质。蛋白质转化为朊病毒通常伴随着β-折叠结构的增加和聚集成寡聚体的倾向。一些朊病毒是有益的，并执行细胞功能，而另一些则导致神经变性。在哺乳动物中，已经鉴定出十几种蛋白质成为朊病毒，在真菌中也发现了类似数量的蛋白质。在哺乳动物和真菌中，朊病毒构象的变化增强了不同朊病毒菌株的生物学特性。越来越多的证据表明，朊病毒引起许多神经退行性疾病（ND），包括阿尔茨海默氏病，帕金森氏病，克雅氏病和卢伽雷氏病，以及tau蛋白病。大多数ND是散发性的，10%至20%是遗传性的。遗传性ND的迟发性，像他们的零星同行，可能反映了随机性质的朊病毒形成，这类疾病的发病机制似乎需要朊病毒积累超过一些关键阈值神经功能障碍的表现。
Prions are proteins that acquire alternative conformations that become self-propagating. Transformation of proteins into prions is generally accompanied by an increase in β-sheet structure and a propensity to aggregate into oligomers. Some prions are beneficial and perform cellular functions, whereas others cause neurodegeneration. In mammals, more than a dozen proteins that become prions have been identified and a similar number has been found in fungi. In both mammals and fungi, variations in the prion conformation encipher the biological properties of distinct prion strains. Increasing evidence argues that prions cause many neurodegenerative diseases (NDs), including Alzheimer’s, Parkinson’s, Creutzfeldt-Jakob, and Lou Gehrig’s diseases, as well as the tauopathies. The majority of NDs are sporadic, and 10% to 20% are inherited. The late onset of heritable NDs, like their sporadic counterparts, may reflect the stochastic nature of prion formation; the pathogenesis of such illnesses seems to require prion accumulation to exceed some critical threshold before neurological dysfunction manifests.
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影响因子：
16.2
作者：
Ash PE;Bieniek KF;Gendron TF;Caulfield T;Lin WL;Dejesus-Hernandez M;van Blitterswijk MM;Jansen-West K;Paul JW 3rd;Rademakers R;Boylan KB;Dickson DW;Petrucelli L
通讯作者：
Petrucelli L
影响因子：
4
作者：
Coskun, Pinar E.;Wyrembak, Joanne;Wallace, Douglas C.
通讯作者：
Wallace, Douglas C.
影响因子：
5.4
作者：
Browning, SR;Mason, GL;Telling, GC
通讯作者：
Telling, GC
影响因子：
4.8
作者：
Baskakov, IV;Legname, G;Cohen, FE
通讯作者：
Cohen, FE
影响因子：
16.2
作者：
de Calignon A;Polydoro M;Suárez-Calvet M;William C;Adamowicz DH;Kopeikina KJ;Pitstick R;Sahara N;Ashe KH;Carlson GA;Spires-Jones TL;Hyman BT
通讯作者：
Hyman BT