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Neuro degeneration with brain Iron accumulation by Mind Map: Neuro degeneration with brain Iron
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Neuro degeneration with brain Iron accumulation

functions of Iron


places in the brain that have most iron


Iron can be toxic


Fe2+ form, picture



NBIA - syndromes

neurodegeneration with brain iron accumuluation, NBIA Type 1, PANK2 gene mutation, formerly: Hallervorden-Spatz disease, Panthotenate kinase associated neurodegeneration type 2, Picture, Madhavi et al 2004, movement disorders society movies, NBIA Type 2, INAD, PLAG2G6, Infantile form, mostly before 2 years of age, ANDA (I), later onset, ANDA (II), Dystonia parkinsonism, Idiopathic form, Paisan-Ruiz et al 2009, Aceruloplasminemia, Neuroferritinopathy, MRI: eye of the tiger sign, Onset 3-5th decade, symptoms, chorea, dystonia, rigidity, seldomly cognitive decline, Hereditary ferritinopathy, onset 3rd decade, Ory-Magne et al 2009, Kufor Rakeb disease, Park 9, Ramirez et al 2006, ATP13A2 mutations, picture

Neurodegenerative disease

Why does iron accumulate?

We don't know

Pantothenat kinase phosphorylation of cystein/pantethin

no phosphorylation


J. Med. Genetics 2009, Gregory et al.

Some indices to look at

hypo intensity in basal ganglia AND hyper intensity