Abnormal Iron Accumulation in Brain and New Iron Chelator for Labile Iron Removal Therapy

Combined Therapy of Iron Chelator and Antioxidant Completely Restores Brain Dysfunction Induced by Iron Toxicity

BACKGROUND Excessive iron accumulation leads to iron toxicity in the brain; however the underlying mechanism is unclear. We investigated the effects of iron overload induced by high iron-diet consumption on brain mitochondrial function, brain synaptic plasticity and learning and memory. Iron chelator (deferiprone) and antioxidant (n-acetyl cysteine) effects on iron-overload brains were also stu...

LPI-labile plasma iron in iron overload.

Labile plasma iron (LPI) represents a component of non-transferrin-bound iron (NTBI) that is both redox-active and chelatable, capable of permeating into organs and inducing tissue iron overload. It appears in various types of hemosiderosis (transfusional and non-transfusional) and in other iron-overload conditions. Sustained levels of LPI could over time compromise organ (e.g. heart) function ...

Neurodegeneration with brain iron accumulation.

Neurodegeneration with brain iron accumulation (NBIA) describes a group of progressive extrapyramidal disorders with radiographic evidence of focal iron accumulation in the brain, usually in the basal ganglia. Patients previously diagnosed with Hallervorden-Spatz syndrome fall into this category. Mutations in the PANK2 gene account for the majority of NBIA cases and cause an autosomal recessive...

Brain and liver iron accumulation in aceruloplasminemia.

A 68-year-old woman with diabetes, anemia, and retinal degeneration developed mild cognitive impairment and subtle gait ataxia. Her parents were nonconsanguineous, and her sister had diabetes. T2*-weighted brain MRI showed symmetric hypointensity in the striatum, thalamus, red nucleus, and dentate nucleus, and on the brain surface, more prominently on high-field imaging (figure 1). Liver MRI de...

Existence of an Erythropoitic labile iron pool