Prion protein protects human neurons against Bax-mediated apoptosis
Prion protein protects human neurons against Bax-mediated apoptosis
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DOI：
10.1074/jbc.c100443200
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发表时间：
2001-10-19
影响因子：
4.8
通讯作者：
LeBlanc, A
中科院分区：
文献类型：
--
作者：
Bounhar, Y;Zhang, Y;LeBlanc, A
细胞朊蛋白（PrP）的功能仍然知之甚少。我们在这里提出了一个前所未有的作用，PrP对ESTA介导的神经元凋亡，并表明，PrP有效地抑制ESTA诱导的细胞死亡在人类初级神经元。缺失PrP的四个八肽重复序列（PrP Delta OR）和家族性D178N和T183A PrP突变完全或部分消除了PrP的神经保护作用。尽管糖基磷脂酰肌醇（GPI）锚信号肽的截短，PrP仍然具有抗凋亡作用，表明PrP的神经保护形式不需要丰富的细胞表面GPI锚定的PrP。我们的研究结果表明，PrP作为一种有效的和新的抗凋亡蛋白对细胞凋亡介导的细胞死亡。
The function of the cellular prion protein (PrP) is still poorly understood. We present here an unprecedented role for PrP against Bax-mediated neuronal apoptosis and show that PrP potently inhibits Bax-induced cell death in human primary neurons. Deletion of four octapeptide repeats of PrP (PrP Delta OR) and familial D178N and T183A PrP mutations completely or partially eliminate the neuroprotective effect of PrP. PrP remains anti-apoptotic despite truncation of the glycosylphosphatidylinositol (GPI) anchor signal peptide, indicating that the neuroprotective form of PrP does not require the abundant cell surface GPI-anchored PrP. Our results implicate PrP as a potent and novel anti-apoptotic protein against Bax-mediated cell death.