Weihl C C, Ghadge G D, Kennedy S G, Hay N, Miller R J, Roos R P
Committee on Neurobiology, University of Chicago, Chicago, Illinois 60637, USA.
J Neurosci. 1999 Jul 1;19(13):5360-9. doi: 10.1523/JNEUROSCI.19-13-05360.1999.
Most early onset cases of familial Alzheimer's disease (AD) are caused by mutations in presenilin-1 (PS1) and presenilin-2 (PS2). These mutations lead to increased beta-amyloid formation and may induce apoptosis in some model systems. Using primary cultured hippocampal neurons (HNs) and rat pheochromocytoma (PC12) cells transiently transfected with replication-defective recombinant adenoviral vectors expressing wild-type or mutant PS1, we demonstrate that mutant PS1s induce apoptosis, downregulate the survival factor Akt/PKB, and affect several Akt/PKB downstream targets, including glycogen synthase kinase-3beta and beta-catenin. Expression of a constitutively active Akt/PKB rescues HNs from mutant PS1-induced neuronal cell death, suggesting a potential therapeutic target for AD. Downregulation of Akt/PKB may be a mechanism by which mutant PS1 induces apoptosis and may play a role in the pathogenesis of familial AD.
大多数早发型家族性阿尔茨海默病(AD)病例是由早老素-1(PS1)和早老素-2(PS2)的突变引起的。这些突变导致β-淀粉样蛋白生成增加,并可能在某些模型系统中诱导细胞凋亡。利用原代培养的海马神经元(HNs)和瞬时转染了表达野生型或突变型PS1的复制缺陷型重组腺病毒载体的大鼠嗜铬细胞瘤(PC12)细胞,我们证明突变型PS1会诱导细胞凋亡,下调存活因子Akt/PKB,并影响多个Akt/PKB下游靶点,包括糖原合酶激酶-3β和β-连环蛋白。组成型活性Akt/PKB的表达可挽救HNs免受突变型PS1诱导的神经元细胞死亡,提示其可能是AD的一个潜在治疗靶点。Akt/PKB的下调可能是突变型PS1诱导细胞凋亡的一种机制,并且可能在家族性AD的发病机制中起作用。