Please use this identifier to cite or link to this item: http://hdl.handle.net/10397/16515
Title: Age-dependent expression of hephaestin in the brain of ceruloplasmin-deficient mice
Authors: Cui, R
Duan, XL
Anderson, GJ
Qiao, YT
Yu, P
Qian, ZM
Yoshida, K
Takeda, S
Guo, P
Yang, ZL
Chang, YZ
Keywords: Ceruloplasmin
Hephaestin
Iron
Iron overload
Issue Date: 2009
Publisher: Elsevier Gmbh, Urban & Fischer Verlag
Source: Journal of trace elements in medicine and biology, 2009, v. 23, no. 4, p. 290-299 How to cite?
Journal: Journal of Trace Elements in Medicine and Biology 
Abstract: Aceruloplasminemia is an autosomal recessive disorder caused by mutations in the ceruloplasmin (CP) gene. It is characterized by iron accumulation in the brain and in visceral organs. However, little is known about the mechanism of iron transport in these regions. Adult CP null (CP-/-) mice show increased iron deposition in several regions of brain, such as the cerebellum and brainstem. In this study, we investigated the expression of the ceruloplasmin homolog hephaestin (Heph) in the brain of CP-/- mice as a function of age. In the cerebral cortex and caudate putamen of 80-week-old CP-/- mice, the expression of Heph increased significantly whilst iron levels remain normal [Patel BN, Dunn RJ, Jeong SY, Zhu Q, Julien JP, David S. Ceruloplasmin regulates iron levels in the CNS and prevents free radical injury. J Neurosci 2002;22(15):6578-6], indicating that Heph might compensate for the loss of CP. In contrast, the substantia nigra and cerebellum of 80-week-old CP-/- mice accumulate iron but do not express high levels or significant decrease of Heph, suggesting that Heph does not replace CP in these regions. These data suggest that Heph may compensate for the loss of CP in a region-specific manner.
URI: http://hdl.handle.net/10397/16515
DOI: 10.1016/j.jtemb.2009.05.004
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