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Neurodegeneration with brain iron accumulation: update on pathogenic mechanisms.


ABSTRACT: Perturbation of iron distribution is observed in many neurodegenerative disorders, including Alzheimer's and Parkinson's disease, but the comprehension of the metal role in the development and progression of such disorders is still very limited. The combination of more powerful brain imaging techniques and faster genomic DNA sequencing procedures has allowed the description of a set of genetic disorders characterized by a constant and often early accumulation of iron in specific brain regions and the identification of the associated genes; these disorders are now collectively included in the category of neurodegeneration with brain iron accumulation (NBIA). So far 10 different genetic forms have been described but this number is likely to increase in short time. Two forms are linked to mut

SUBMITTER: Levi S 

PROVIDER: S-EPMC4019866 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature