α-晶状体蛋白对维持晶状体透明性的功能研究进展

Functional research advances of the protection of alpha crystallin to the lens transparency

α-晶状体蛋白是眼晶状体细胞质的主要成分,由小热休克蛋白家族中的αA-晶状体蛋白和αB-晶状体蛋白组成.α-晶状体蛋白具有分子伴侣活性,还有调控细胞周期、增强基因组稳定性、防止压力诱导性细胞凋亡的作用.α-晶状体蛋白相关基因突变常引起遗传性白内障,是目前儿童盲的主要原因,而α-晶状体蛋白的多种改变可导致年龄相关性白内障.了解α-晶状体蛋白的功能有助于理解晶状体的发育及其正常功能的维持,为预防及治疗α-晶状体蛋白相关性白内障提供理论依据.就白内障发生机制、遗传定位及晶状体蛋白的功能进行综述.

α-Crytallin,composed of two menbers of the small heat shock protein,is the major component of the cytoplasm in lens.α-Crytallin not only possesses chaperone-like activity,but also plays important roles in regulation of cell cycle,enhancing genome stability and prevention of the stress-induced apoptosis.The gene mutation associated with α-crytallin is a common cause of hereditary cataract,which is the major cause of childhood blindness.Whereas various changes of the α-crytallin can result in the most common cause of blindness in the world—age-related cataract.Understanding of the function of α-crytallin helps to comprehend the development of lens and how the lens maintain its normal function,and to provide a theoretical basis for the prevention and treatment of the cataract related to the malfunction of α-crytallin.The genetic mapping and mechanism of cataract and the function of α-crytallin are summarized here.