Q J Med 2001; 94: 417-422
© 2001 Association of Physicians
Increased oxysterols associated with iron accumulation in the brains and visceral organs of acaeruloplasminaemia patients
From the First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu, 1 Department of Legal Medicine, Kobe University School of Medicine, Kobe, and 2 Kobe Pharmaceutical University, Kobe, Japan
Received 19 September 2000 and in revised form 23 May 2001
Acaeruloplasminaemia is characterized by excessive neurovisceral iron accumulation due to mutation of the caeruloplasmin gene. Excess iron functions as a potent catalyst of biological oxidation, and increased iron concentration is associated with the products of lipid peroxidation in the serum and cerebrospinal fluid. We investigated whether the amount of iron accumulated paralleled lipid peroxidation levels in acaeruloplasminaemia tissues, examining brains and visceral organs of two affected patients at autopsy for iron and copper content, and oxysterols, including 7-hydroxycholesterol and 7-ketocholesterol, which are directly produced from cholesterol by active oxygen species. The amount of iron accumulated in various tissues was correlated with the levels of the oxysterols. These findings suggest that lipid peroxidation produced by the intracellular accumulation of iron is involved in the pathogenesis of acaeruloplasminaemia.
Address correspondence to Dr H. Miyajima, The First Department of Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Hamamatsu 431-3192, Japan. e-mail: miyajima{at}hama\|[hyphen]\|med.ac.jp
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