國家衛生研究院 NHRI:Item 3990099045/5811
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    题名: Alpha1-antitrypsin deficiency and conformational diseases
    其它题名: α1-抗胰蛋白酶缺乏症與蛋白質構型變異疾病
    作者: Chang, YP;Chang, WS;W. Chu, YH
    贡献者: National Institute of Cancer Research
    摘要: Abnormal protein aggregation is responsible for numerous diseases such as Alzheimer’s disease, Parkinson’s disease and α1-antitrypsin deficiency (AATD). This category of diseases is now recognized as conformational diseases. α1-Antitrypsin is the archetype of the serine protease inhibitor (serpin) superfamily, and its physiological role is a suicide substrate toward its cognate enzyme neutrophil elastase. Through an unique conformational transition, the snared elastase is neutralized and the alveolar matrices in lungs are protected from destruction. However, dysfunctional mutations can result in a loss of inhibitory activity or allow spontaneous and inappropriate intermolecular polymerization ofα1-antitrypsin, ultimately leading to emphysema and cirrhosis. Insights of the mechanism of diseases offer a promising anti-protein polymerization strategy. Previous studies have shown that synthetic peptides can anneal to Zα1-antitrypsin and block polymerization. Most recently, we have developed a combinatorial approach for the discovery of anti-protein polymerization ligands, valuable for the design of therapeutic agents
    日期: 2005-09
    關聯: 化學. 2005 Sep;63(3):419-430.
    Link to: http://readopac1.ncl.edu.tw/nclJournal/search/detail.jsp?sysId=0005798517&dtdId=000040&search_type=detail&la=ch
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