Chaperonopathies and chaperonotherapy

被引:59
作者
Macario, Alberto J. L. [1 ]
de Macario, Everly Conway [1 ]
机构
[1] Univ Maryland, Inst Biotechnol, Columbus Ctr, Baltimore, MD 21202 USA
关键词
chaperonology; chaperonopathies; chaperonotherapy; stress; protein misfolding; protein aggregation;
D O I
10.1016/j.febslet.2007.04.030
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
The study of molecular chaperones (genetics, structure, location, physiology, pathology, and therapeutics) has developed into a science with specific objectives, methods, and hypotheses, a discipline we called chaperonology. Subdisciplines of chaperonology include the study of pathological chaperones (chaperonopathies) and the analysis of their genes in sequenced genomes (chaperonomics). Cbaperonopathies are pathological conditions in which one type of chaperone is deficient due to a genetic or acquired defect that modifies the chaperone's structure and/or makes the chaperone unavailable for functioning when needed. Experimental and clinical data show that chaperones and their genes can be used for treating various pathological conditions, thus justifying the development of chaperonotherapy. We discuss recent work showing that chaperonotherapy is on solid foundations: the data demonstrate that molecular chaperones counteract pathogenetic mechanisms in disease and during stress. (c) 2007 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.
引用
收藏
页码:3681 / 3688
页数:8
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