Glucose and oxygen hypometabolism in aceruloplasminemia brains

Internal Medicine
H MiyajimaYasuomi Ouchi

Abstract

Aceruloplasminemia is an iron metabolic disorder caused by mutations in the ceruloplasmin gene. It is characterized by progressive neurodegeneration in association with iron accumulation. Excess iron functions as a potent catalyst of biologic oxidation. Previously we showed that an increased iron concentration is associated with the products of lipid peroxidation in the serum, cerebrospinal fluid, and brain tissues. To clarify the free radical-mediated tissue injury caused by intracellular iron accumulation through mitochondrial dysfunction. We have measure brain oxygen and glucose metabolisms using positron emission tomography (PET) and examined brains at autopsy for iron contents and activities of the mitochondrial respiratory chain in two affected patients who had different truncation mutations of the ceruloplasmin gene. PET showed a marked decrease in glucose and oxygen consumption in the entire brain of aceruloplasminemia patients, with a preponderance of metabolic reduction in basal ganglia. Enzyme activities in the mitochondrial respiratory chain of the basal ganglia were reduced to approximately 45% and 42% respectively for complexes I and IV. An inverse relationship was shown between the amounts of iron accumulated and...Continue Reading

Citations

Mar 15, 2005·Journal of Neurology·Hiroaki MiyajimaMasanobu Sakamoto
Jul 10, 2003·Lancet Neurology·Ya Ke, Zhong Ming Qian
Feb 20, 2008·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Luis F Gonzalez-CuyarRudy J Castellani
Oct 20, 2012·Toxicology and Applied Pharmacology·Zivanit ErgazAsher Ornoy
Sep 18, 2007·Progress in Neurobiology·Ya Ke, Zhong Ming Qian
Jul 3, 2004·Ageing Research Reviews·Hyman M Schipper
Sep 25, 2021·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Maria SalsoneAldo Quattrone

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