The pathological deposition of excess iron within specific regions of the central nervous system, particularly in the basal ganglia, which can lead to neurodegeneration and functional impairment. Iron is essential for oxygen transport and cellular respiration, but its dysregulated accumulation promotes oxidative stress by catalyzing the formation of reactive oxygen species. This condition is often observed in certain genetic disorders and is increasingly recognized as a factor in age-related neurological decline.
Origin
This term is rooted in neuropathology and neurochemistry, specifically concerning the metabolism of transition metals within the brain parenchyma. Clinical recognition stems from advanced magnetic resonance imaging (MRI) techniques that can quantify iron deposits, and the identification of genetic syndromes like Neurodegeneration with Brain Iron Accumulation (NBIA).
Mechanism
The mechanism involves a failure in iron homeostasis, where transport proteins like transferrin and ferritin, along with regulatory hormones like hepcidin, become dysfunctional within the brain’s unique microenvironment. Excess iron in a free or loosely bound state participates in the Fenton reaction, generating highly damaging hydroxyl radicals. This oxidative damage compromises neuronal membrane integrity and contributes to the pathogenesis of neurodegenerative diseases.
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