<正>1993年,Wadhwa等[1]在小鼠胚胎成纤维细胞(mouse embryonic fibroblasts,MEF)中首次克隆出了Mortalin(mtHSP70/HSP75/Grp75/TRAP-1/PBP74),它是热休克蛋白70(heat-shock protein 70,HSP70)家族成员之一。研究表明,Mortalin参与了多种生理功能的调控,包括细胞存活、细胞增殖、压力应激、线粒体合成、细胞内转运等,并且可能在神经系统疾病中发挥作用。在此,本文就Mortalin的基本结构、生
Mortalin(mtHSP70/HSP75/Grp75/TRAP -1/PBP74) is an essential mitoehondrial chaperone and is believed to regulate multiple cellular functions ranging from cell survival, cell proliferation, stress response, mitochondri- al biogenesis and intraeellular trafficking. Recent studies have shown that mortalin is relevant to the pathogenesis of Alzheimer's disease( A D ) and Parkinson's disease (PD). Proteomic studies demonstrate the difftrential expression of mortalin not only in the models of AD or PD but also in PD human brains as compared to the controls. Mortalin seems to modu- lates the development of diseases through association with the critical molecules such as apoE, amyloid β - protein, amyloid precursor protein(APP) in AD, and α -synuclein, DJ - 1, PINK1 in PD. Mortalin may regulate PD development via the pathways involving mitoehondrial and proteasomal functions as well as oxidative stress. Furthermore, it is reported that mortalin participates in the pathological processes of several other neurological disorders, such as ischemic brain injury, ab- sence seizure and Friedreich's ataxia(FRDA).