A novel neuroferritinopathy mouse model (FTL 498InsTC) shows progressive brain iron dysregulation, morphological signs of early neurodegeneration and motor coordination deficits

A novel neuroferritinopathy mouse model (FTL 498InsTC) shows progressive brain iron dysregulation, morphological signs of early neurodegeneration and motor coordination deficits

Neuroferritinopathy is a rare genetic disease with a dominant autosomal transmission caused by mutations of the ferritin light chain gene (FTL). It belongs to Neurodegeneration with Brain Iron Accumulation, a group of disorders where iron dysregulation is tightly associated with neurodegeneration. W...

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Bibliographic Details
Main Authors: Federica Maccarinelli, Antonella Pagani, Anna Cozzi, Franca Codazzi, Giuseppina Di Giacomo, Sara Capoccia, Stefania Rapino, Dario Finazzi, Letterio Salvatore Politi, Francesca Cirulli, Marco Giorgio, Ottavio Cremona, Fabio Grohovaz, Sonia Levi
Format: Article
Language:English
Published: Elsevier 2015-09-01
Series:Neurobiology of Disease
Subjects:
Neuroferritinopathy
Neurodegenerative disorder
Ferritin
Iron
Oxidative damage
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996114003362

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