Abstract：
:Two decades ago, patients lacking circulating serum ceruloplasmin (Cp) presented with neurodegeneration associated with brain iron accumulation. These patients, with mutations in the MCO (multi-copper oxidase), Cp, revealed an essential role for Cp in iron homoeostasis. The patients were diagnosed in adulthood with CNS (central nervous system) disease and progressed rapidly, making understanding the mechanism of disease imperative. We now know that (i) Cp regulates the efficiency of iron efflux, (ii) Cp stabilizes ferroportin membrane expression, (iii) GPI (glycosylphosphatidylinositol)-linked Cp is the predominant form expressed in brain, (iv) Cp functions as a ferroxidase and regulates the oxidation of Fe(2+) to Fe(3+), (v) Cp does not bind to transferrin directly, and (vi) Cp is one member of a family of mammalian MCOs, which includes hephaestin. It is still unclear how an absence of Cp results in neurodegeneration: is the iron accumulation a primary or secondary injury? Although it is attractive to invoke an iron-mediated oxidative stress mechanism for the neuronal injury and degeneration in aceruloplasminaemia, our data suggest limited redox injury in the brains of mice lacking MCO. In fact, we propose a role for neuronal iron starvation with associated astrocyte and microglial iron overload. With the defect in aceruloplasminaemia being one of inefficient iron efflux from macrophages, we believe that the iron is trapped in a compartment not readily available to participate in oxyradical injury. It is likely that different mechanisms of neuronal cell protection are offered by astrocytes and microglia, and, once these cells are damaged, neuronal survival is compromised.
journal_name
Biochem Soc Transjournal_title
Biochemical Society transactionsauthors
Texel SJ,Xu X,Harris ZLdoi
10.1042/BST0361277subject
Has Abstractpub_date
2008-12-01 00:00:00pages
1277-81issue
Pt 6eissn
0300-5127issn
1470-8752pii
BST0361277journal_volume
36pub_type
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