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我们的身体如何对抗淀粉样变性:生理因素对淀粉样蛋白致病性聚集的影响。

How our bodies fight amyloidosis: effects of physiological factors on pathogenic aggregation of amyloidogenic proteins.

作者信息

Huang Lianqi, Liu Xinran, Cheng Biao, Huang Kun

机构信息

Tongji School of Pharmacy, Huazhong University of Science and Technology, Wuhan, Hubei 430030, PR China.

Department of Pharmacy, Central Hospital of Wuhan, Wuhan, Hubei 430014, PR China.

出版信息

Arch Biochem Biophys. 2015 Feb 15;568:46-55. doi: 10.1016/j.abb.2015.01.007. Epub 2015 Jan 20.

Abstract

The process of protein aggregation from soluble amyloidogenic proteins to insoluble amyloid fibrils plays significant roles in the onset of over 30 human amyloidogenic diseases, such as Prion disease, Alzheimer's disease and type 2 diabetes mellitus. Amyloid deposits are commonly found in patients suffered from amyloidosis; however, such deposits are rarely seen in healthy individuals, which may be largely attributed to the self-regulation in vivo. A vast number of physiological factors have been demonstrated to directly affect the process of amyloid formation in vivo. In this review, physiological factors that influence amyloidosis, including biological factors (chaperones, natural antibodies, enzymes, lipids and saccharides) and physicochemical factors (metal ions, pH environment, crowding and pressure, etc.), together with the mechanisms underlying these proteostasis effects, are summarized.

摘要

从可溶性淀粉样蛋白生成蛋白到不溶性淀粉样纤维的蛋白质聚集过程在30多种人类淀粉样变性疾病的发病中起重要作用,如朊病毒病、阿尔茨海默病和2型糖尿病。淀粉样沉积物常见于患有淀粉样变性的患者中;然而,在健康个体中很少见到这种沉积物,这可能在很大程度上归因于体内的自我调节。大量生理因素已被证明直接影响体内淀粉样蛋白形成过程。在本综述中,总结了影响淀粉样变性的生理因素,包括生物因素(伴侣蛋白、天然抗体、酶、脂质和糖类)和物理化学因素(金属离子、pH环境、拥挤和压力等),以及这些蛋白质稳态效应的潜在机制。

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