Increased lipid peroxidation in the brains of aceruloplasminemia patients

• Published in: Journal of the neurological sciences Vol. 175; no. 2; pp. 91 - 95
• Main Authors: Yoshida, Kunihiro, Kaneko, Kazuma, Miyajima, Hiroaki, Tokuda, Takahiko, Nakamura, Akinori, Kato, Masahiro, Ikeda, Shu-ichi
• Format: Journal Article
• Language: English
• Published: Shannon Elsevier B.V 15.04.2000; Elsevier Science
• Subjects: Aceruloplasminemia; Aged; Aged, 80 and over; Aldehydes - metabolism; Biological and medical sciences; Brain - metabolism; Brain - pathology; Ceruloplasmin; Ceruloplasmin - deficiency; Hemosiderosis - metabolism; Humans; Immunohistochemistry; Lipid Peroxidation; Male; Malondialdehyde - metabolism; Medical sciences; Metabolic diseases; Metals (hemochromatosis...); Middle Aged; Other metabolic disorders; Oxidative stress; Ceruloplasmin; Lipid peroxidation; Oxidative stress; Aceruloplasminemia; Human; Immunohistochemistry; Pathology; Central nervous system; Metabolic diseases; Exploration; Lipids; Genetic disease; Brain (vertebrata); Peroxidation
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/S0022-510X(00)00295-1

Aceruloplasminemia is characterized by excessive neurovisceral accumulation of iron due to mutations in the ceruloplasmin gene. We report that the levels of malonaldehyde (MDA) and 4-hydroxynonenals (4-HNE) were elevated both in the frontal cortex and putamen in two patients with aceruloplasminemia compared with those in controls. Immunohistochemical analysis with an antibody raised against HNE-modified protein showed a large number of immunoreactive neurons and glias in the brain of a patient. MDA and 4-HNE are good indicators of lipid peroxidation because they are generated in the process of oxidation of polyunsaturated fatty acids. The data strongly suggest that the increase of oxygen free radicals associated with excessive iron accumulation plays an important pathogenic role in the process of neuronal cell death in the brains of patients with aceruloplasminemia.