Increased Lipid Peroxidation and Mitochondrial Dysfunction in Aceruloplasminemia Brains

ABSTRACT Aceruloplasminemia is characterized by iron accumulation in the brain as well as in visceral organs, due to the absence of ceruloplasmin ferroxidase activity. The neurological symptoms, which include involuntary movements, ataxia, and dementia, reflect the sites of iron deposition. The uniq...

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Bibliographic Details
Published inBlood cells, molecules, & diseases Vol. 29; no. 3; pp. 433 - 438
Main Authors Miyajima, Hiroaki, Kono, Satoshi, Takahashi, Yoshitomo, Sugimoto, Masahiro
Format Journal Article
LanguageEnglish
Published United States Elsevier Inc 01.11.2002
Subjects
4-hydroxynonenal
Aged
Brain - diagnostic imaging
Brain - physiopathology
ceruloplasmin
Ceruloplasmin - deficiency
Electron Transport - physiology
Female
Free Radicals - metabolism
Humans
iron
Lipid Peroxidation
Male
malondialdehyde
Middle Aged
Mitochondria - pathology
Mitochondria - physiology
mitochondrial respiratory chain
Oxidative Stress - physiology
Radiography
ceruloplasmin
iron
mitochondrial respiratory chain
malondialdehyde
4-hydroxynonenal
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Summary:ABSTRACT Aceruloplasminemia is characterized by iron accumulation in the brain as well as in visceral organs, due to the absence of ceruloplasmin ferroxidase activity. The neurological symptoms, which include involuntary movements, ataxia, and dementia, reflect the sites of iron deposition. The unique involvement of the central nervous system distinguishes aceruloplasminemia from other inherited and acquired iron storage disorders. Excess iron functions as a potent catalyst of biologic oxidation. An increased iron concentration was associated with increased lipid peroxidation in the brains of three aceruloplasminemia patients. Positron emission tomography showed brain glucose and oxygen hypometabolism. Enzyme activities in the mitochondrial respiratory chain of the basal ganglia were reduced to about 50 and 43%, respectively, for complexes I and IV. Those of the cerebral and cerebellar cortices also were decreased approximately 62 and 65%. These findings suggest that iron-mediated free radicals may contribute to neuronal cell damage through increased lipid peroxidation and the impairment of mitochondrial energy metabolism in aceruloplasminemia brains.
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ISSN:1079-9796
1096-0961
DOI:10.1006/bcmd.2002.0561