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Excessive accumulation of iron in the brain
Patients with certain neurodegenerative disorders such as Parkinson's disease and dementia show excessive accumulation of iron in the brain. It has been postulated that these iron deposits damage neurons by inducing oxidative stress, but whether they are a cause or consequence of the disease process is unclear.
Curtis et al. report that the causative mutation in a family with adult-onset basal ganglia disease lies in a gene encoding a subunit of ferritin, a protein that functions in both storage and detoxification of iron. In a study of patients with Hallervorden-Spatz syndrome, an early-onset neurodegenerative disorder, Zhou et al. find that the culprit gene encodes pantothenate kinase (PANK), an essential regulatory enzyme in coenzyme A biosynthesis. The disease-associated mutations in PANK might alter iron levels in the brain indirectly through effects on cysteine levels. Further studies of these new disease genes will be needed to understand the role iron metabolism plays in neurodegeneration.
Nature Genet. 10.1038/ng571; 10.1038/ng572.
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