Brain is the one of the most energy-demanding tissues in our body. This means it requires a high amount of iron, one of the necessary factors for making energy. Iron is supplied to the brain through the blood. Currently, it is not clear how the body controls the amount of iron delivered to the brain to maintain the appropriate level. Too much iron will cause tissue damage, and too little iron will keep cells from working normally.
NBIA contains number of different diseases with some common features, including misregulated iron accumulated in patients’ brains. Although it has been known for many years that PKAN, the most common NBIA, is caused by PANK2 mutations, the link to iron metabolism has been a mystery. New experiments led by Dr. Suh Young Jeong will study how mutations in PANK2 cause iron accumulation in brain tissue and lead to cell death.
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