Laskowska Ewa
Katedra Biochemii, Instytut Biologii, Uniwersytet Gdański, ul. Kładki 24, 80-952 Gdańsk.
Postepy Biochem. 2007;53(1):19-26.
Small heat shock proteins (sHsps) belong to molecular chaperones, which protect prokaryotic and eukaryotic cells against deleterious effects, of stress. sHsps prevent stress induced, irreversible aggregation of damaged proteins and facilitate renaturation of bound substrates cooperating with other molecular chaperones. This review summarizes recent studies focused mainly on the involvement of sHsps in diseases related to protein aggregation. sHsps are often a component of protein aggregates forming during progress of neurodegenerative disorders. Mutation in sHsps genes have been identified, which are responsible for development of cataract, desmin related myopathy and neuropathies. sHsps protect cells against oxidative stress resulting from ischemia/reperfusion during heart or brain stroke. Several studies indicate that sHsp participate in regulation of apoptosis and are involved in cancerogenesis. Uncovering the sHsps role in diseases enable to develop new therapeutic strategies.
小分子热休克蛋白(sHsps)属于分子伴侣,可保护原核细胞和真核细胞免受应激的有害影响。sHsps可防止应激诱导的受损蛋白质不可逆聚集,并与其他分子伴侣协同促进结合底物的复性。本综述总结了近期主要聚焦于sHsps参与与蛋白质聚集相关疾病的研究。sHsps通常是神经退行性疾病进展过程中形成的蛋白质聚集体的组成部分。已鉴定出sHsps基因中的突变,这些突变与白内障、结蛋白相关肌病和神经病变的发生有关。sHsps可保护细胞免受心脏或脑卒中等缺血/再灌注引起的氧化应激。多项研究表明,sHsp参与细胞凋亡的调节并与肿瘤发生有关。揭示sHsps在疾病中的作用有助于开发新的治疗策略。