Back arching was reported in one of the very first patients with neurodegeneration with brain iron accumulation syndrome (NBIAs) published in 1936. However, recent reports have mainly focused on the genetic and imaging aspects of these disorders, and the phenotypic characterization of the dystonia has been lost. In evaluating patients with NBIAs in our centers, we have observed that action-indu...

The capacity to act as an electron donor and acceptor makes iron an essential cofactor of many vital processes. Its balance in the body has to be tightly regulated since its excess can be harmful by favouring oxidative damage, while its deficiency can impair fundamental activities like erythropoiesis. In the brain, an accumulation of iron or an increase in its availability has been associated w...

Backgrounds: It remains unclear whether radiation-induced haemorrhage in the spleen causes iron accumulation, and subsequently, ferroptosis in splenic lymphocytes. In this study, we investigated the occurrence of ferroptosis in splenic lymphocytes of gamma-irradiated mice. Materials and Methods: Mice were subjected to gamma radiation from a 137Cs source. Iron, Ferroportin 1, and iron regulatory...

Dysfunction of the ubiquitin-proteasome system (UPS) and accumulation of iron in substantia nigra (SN) are implicated in the pathogenesis of Parkinson's disease (PD). UPS dysfunction and iron misregulation may reinforce each other's contribution to the degeneration of dopamine (DA) neurons. In the present study, we use a new brain-permeable iron chelator, VK-28 [5-(4-(2-hydroxyethyl) piperazin-...

BACKGROUND AND PURPOSE Experimental studies have suggested a role for iron accumulation in the pathology of TBI. Magnetic field correlation MR imaging is sensitive to the presence of non-heme iron. The aims of this study are to 1) assess the presence, if any, and the extent of iron deposition in the deep gray matter and regional white matter of patients with mTBI by using MFC MR imaging; and 2)...

An accurate assessment of iron accumulation is required for diagnosis and therapy of iron overload in various neurodegenerative diseases. Susceptibility Weighted Imaging (SWI) offers information about any tissue that has a different susceptibility than its surrounding structures. Reliable methods to precisely quantify brain iron are essential. Image segmentation refers to partition of an image ...

Recent studies have postulated a role for vitamin D and its receptor on cerebral function, and anti-inflammatory, immunomodulatory and neuroprotective effects have been described; vitamin D can inhibit proinflammatory cytokines and nitric oxide synthesis during various neurodegenerative insults, and may be considered as a potential drug for the treatment of these disorders. In addition, iron is...

Elevated iron accumulation has been reported in brain regions in some neurodegenerative disorders. However, the mechanism for this is largely unknown. Divalent metal transporter 1 (DMT1) is an important divalent cation transporter. The aim of the present study is to construct recombinant adenovirus encoding human DMT1 with iron responsive element (DMT1+IRE) and infect MES23.5 dopaminergic cells...

The role of iron in the pathogenesis of Alzheimer's disease (AD) is still unclear, despite the evidence that it accumulates in the same brain regions characterized by the amyloid beta peptide (Abeta) accumulation. Here we propose that iron directly influences the Abeta production through the modulation of furin, a proconvertase involved in the regulation of the alpha-secretase-dependent process...

Acaeruloplasminaemia is a rare neurodegenerative disorder in which dysfunction of the caeruloplasmin gene results in brain iron accumulation alongside systemic iron overload. We discuss the case of a 53-year-old male who presented to neurology with a 4-year history of progressive neuropsychiatric symptoms and was eventually diagnosed with this condition. We discuss the characteristic clinical f...