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Pharmaceuticals 2019, 12(1), 27; https://doi.org/10.3390/ph12010027

Neurodegeneration with Brain Iron Accumulation Disorders: Valuable Models Aimed at Understanding the Pathogenesis of Iron Deposition

1
School of Medicine, University Vita-Salute San Raffaele, 20132 Milano, Italy
2
San Raffaele Scientific Institute, Division of Neuroscience, 20132 Milano, Italy
3
Fondazione IRCCS Istituto Neurologico Carlo Besta, 20126 Milano, Italy
*
Author to whom correspondence should be addressed.
Received: 19 December 2018 / Revised: 25 January 2019 / Accepted: 29 January 2019 / Published: 9 February 2019
(This article belongs to the Special Issue Iron as Therapeutic Targets in Human Diseases)
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Abstract

Neurodegeneration with brain iron accumulation (NBIA) is a set of neurodegenerative disorders, which includes very rare monogenetic diseases. They are heterogeneous in regard to the onset and the clinical symptoms, while the have in common a specific brain iron deposition in the region of the basal ganglia that can be visualized by radiological and histopathological examinations. Nowadays, 15 genes have been identified as causative for NBIA, of which only two code for iron-proteins, while all the other causative genes codify for proteins not involved in iron management. Thus, how iron participates to the pathogenetic mechanism of most NBIA remains unclear, essentially for the lack of experimental models that fully recapitulate the human phenotype. In this review we reported the recent data on new models of these disorders aimed at highlight the still scarce knowledge of the pathogenesis of iron deposition. View Full-Text
Keywords: iron; neurodegeneration; NBIA iron; neurodegeneration; NBIA
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Levi, S.; Tiranti, V. Neurodegeneration with Brain Iron Accumulation Disorders: Valuable Models Aimed at Understanding the Pathogenesis of Iron Deposition. Pharmaceuticals 2019, 12, 27.

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