McCaig Colin D
Institute of Medical Sciences, University of Aberdeen, Aberdeen, Scotland, UK.
Rev Physiol Biochem Pharmacol. 2025;187:195-237. doi: 10.1007/978-3-031-68827-0_13.
Ribosomes use multiple electrical forces to regulate new protein construction, to ensure efficient protein cotranslation, chaperoning, and folding. When these electrical regulatory forces are disrupted as in point charge mutations, specific disease occurs from aberrantly folded proteins. α1 antitrypsin deficiency is perhaps the best-known misfolded protein disease and is covered in some detail.
核糖体利用多种电力来调节新蛋白质的构建,以确保高效的蛋白质共翻译、分子伴侣作用和折叠。当这些电调节力像在点电荷突变中那样受到干扰时,异常折叠的蛋白质会引发特定疾病。α1抗胰蛋白酶缺乏症可能是最广为人知的错误折叠蛋白疾病,本文将对此进行详细阐述。