Abstract：
:Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late-onset neurodegenerative disorder associated with premutation alleles of the FMR1 gene that is characterized by progressive action tremor, gait ataxia, and cognitive decline. Recent studies of mitochondrial dysfunction in FXTAS have suggested that iron dysregulation may be one component of disease pathogenesis. We tested the hypothesis that iron dysregulation is part of the pathogenic process in FXTAS. We analyzed postmortem choroid plexus from FXTAS and control subjects, and found that in FXTAS iron accumulated in the stroma, transferrin levels were decreased in the epithelial cells, and transferrin receptor 1 distribution was shifted from the basolateral membrane (control) to a predominantly intracellular location (FXTAS). In addition, ferroportin and ceruloplasmin were markedly decreased within the epithelial cells. These alterations have implications not only for understanding the pathophysiology of FXTAS, but also for the development of new clinical treatments that may incorporate selective iron chelation.
journal_name
Brain Resjournal_title
Brain researchauthors
Ariza J,Steward C,Rueckert F,Widdison M,Coffman R,Afjei A,Noctor SC,Hagerman R,Hagerman P,Martínez-Cerdeño Vdoi
10.1016/j.brainres.2014.11.058subject
Has Abstractpub_date
2015-02-19 00:00:00pages
88-96eissn
0006-8993issn
1872-6240pii
S0006-8993(14)01665-5journal_volume
1598pub_type
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