Copper chelation delays the onset of prion disease
Copper chelation delays the onset of prion disease
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DOI：
10.1074/jbc.c300303200
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发表时间：
2003-11-21
影响因子：
4.8
通讯作者：
Wisniewski, T
中科院分区：
文献类型：
--
作者：
Sigurdsson, EM;Brown, DR;Wisniewski, T
朊病毒蛋白（PrP）结合铜，在某些条件下，铜可以促进其折叠成更具蛋白酶抗性的形式。因此，铜水平可能会影响羊瘙痒症形式的朊病毒蛋白（PrPSc）的感染性。为了确定铜靶向治疗朊病毒病的可行性，我们用铜螯合剂D-（-）-青霉胺（D-PEN）治疗小鼠，在腹腔内接种瘙痒症后立即开始。D-PEN使小鼠朊病毒病的发病时间推迟了约11天（p=0.002），并使脑中铜水平降低了29%（p
The prion protein (PrP) binds copper and under some conditions copper can facilitate its folding into a more protease resistant form. Hence, copper levels may influence the infectivity of the scrapie form of prion protein (PrPSc). To determine the feasibility of copper-targeted therapy for prion disease, we treated mice with a copper chelator, D-(-)- penicillamine (D-PEN), starting immediately following intraperitoneal scrapie inoculation. D-PEN delayed the onset of prion disease in the mice by about 11 days (p=0.002), and reduced copper levels in brain by 29% (p