Poor regulation of iron metabolism is seen in some patients with neurodegenerative diseases, such as Parkinson’s disease (PD), Alzheimer’s disease (AD), and amyotrophic lateral sclerosis (ALS). Iron transport in and out of brain cells helps maintain cellular balance. Imbalance in iron levels produces neurotoxins, reactive oxygen species and oxidative stress. Iron accumulation in the cells in AD and PD is believed to occur from the loss of iron balance. In ALS, studies show abnormalities in genes related to increased iron uptake, oxidative stress and abnormal inflammatory response.
There are various iron chelators which reduce iron stores in the brain and are neuroprotective in neurodegenerative diseases. They reduce apoptosis of brain cells and protect against oxidative stress, possibly by removing the excess iron. One such chelator is desferrioxamine (DFO). This is of limited benefit because of short half-life, inability to cross the blood-brain barrier, and side effects.
Deferiprone (DFP) is an oral iron chelating agent used to treat thalassemia (a blood disease). DFP does remove iron from the brain in Friedreich’s ataxia. Side effects of DFP include bone marrow problems and liver damage. A combination of DFO and DFP has shown benefit in thalassemia. Several new synthetic iron chelating agents have been developed.
CONCLUSION: Imbalance of iron regulation in the brain is seen in Parkinson’s disease, Alzheimer’s disease and amyotrophic lateral sclerosis (ALS). Iron chelators to remove excess iron are being studied in order to treat a variety of neurodegenerative diseases.
NOTE: It should be noted that body fluids contain a chemical called lactoferrin, which is an iron chelator. It is available as a supplement.
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