Ceruloplasmin dysfunction and therapeutic potential for Parkinson disease

被引：234

作者：

Ayton, Scott ^{[1
]}

Lei, Peng ^{[1
]}

Duce, James A. ^{[1
]}

Wong, Bruce X. W. ^{[1
]}

Sedjahtera, Amelia ^{[1
]}

Adlard, Paul A. ^{[2
,3
]}

Bush, Ashley I. ^{[1
,3
]}

Finkelstein, David I. ^{[1
,2
]}

机构：

[1] Univ Melbourne, Florey Inst Neurosci & Mental Hlth, Oxidat Biol Lab, Parkville, Vic, Australia

[2] Univ Melbourne, Florey Inst Neurosci & Mental Hlth, Parkville, Vic, Australia

[3] Univ Melbourne, Dept Pathol, Parkville, Vic, Australia

来源：

ANNALS OF NEUROLOGY | 2013年 / 73卷 / 04期

基金：

澳大利亚研究理事会; 英国医学研究理事会;

关键词：

NIGRAL IRON DEPOSITION; FERROXIDASE ACTIVITY; SUPEROXIDE-DISMUTASE; LIPID-PEROXIDATION; SUBSTANTIA-NIGRA; SERUM; GENE; NEURONS; COPPER; EXPORT;

D O I：

10.1002/ana.23817

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Ceruloplasmin is an iron-export ferroxidase that is abundant in plasma and also expressed in glia. We found a approximate to 80% loss of ceruloplasmin ferroxidase activity in the substantia nigra of idiopathic Parkinson disease (PD) cases, which could contribute to the pro-oxidant iron accumulation that characterizes the pathology. Consistent with a role for ceruloplasmin in PD etiopathogenesis, ceruloplasmin knockout mice developed parkinsonism that was rescued by iron chelation. Additionally, peripheral infusion of ceruloplasmin attenuated neurodegeneration and nigral iron elevation in the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine mouse model for PD. These findings show, in principle, that intravenous ceruloplasmin may have therapeutic potential in PD. Ann Neurol 2013;73:554-559

引用

页码：554 / 559

页数：6

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