A growing number of neurodegenerative conditions, such as sporadic Creutzfeldt-Jakob disease (sCJD), Alzheimer’s disease (AD), and Parkinson’s disease (PD), are associated with accumulation of iron in the brain. The underlying cause is complex and only partially known. A better understanding of whether iron plays a primary or secondary role in disease pathogenesis is desirable and is likely to ...

Mutations in PANK2 lead to neurodegeneration with brain iron accumulation. PANK2 has a role in the biosynthesis of coenzyme A (CoA) from dietary vitamin B5, but the neuropathological mechanism and reasons for iron accumulation remain unknown. In this study, atypical patient-derived fibroblasts were reprogrammed into induced pluripotent stem cells (iPSCs) and subsequently differentiated into cor...

Manganese (Mn) is an essential metal required for normal homeostasis. Humans chronically exposed to high Mn levels, however, may exhibit psychomotor signs secondary to increased brain Mn. As Mn and iron (Fe) share several cellular membrane transporters, decreased Fe levels resulting from Fe deficiency or anemia lead to increased brain Mn deposition. Conversely, decreased Mn levels are associate...

Ferritin is the main intracellular protein of iron storage with a central role in the regulation of iron metabolism and detoxification. Nucleotide insertions in the last exon of the ferritin light chain cause a neurodegenerative disease known as Neuroferritinopathy, characterized by iron deposition in the brain, particularly in the cerebellum, basal ganglia and motor cortex. The disease progres...

BACKGROUND Iron accumulation in the central nervous system (CNS) is a common feature of many neurodegenerative diseases. Multicopper ferroxidases (MCFs) play an important role in cellular iron metabolism. However, the role of MCFs in the CNS in health and disease remains poorly characterized. OBJECTIVE The aim was to study the role of hephaestin (HEPH) and ceruloplasmin (CP) in CNS iron metab...

opDivision qf' Phauaacognosy, Department of' JdbdiclnaJ Resources, institute of Natural Medicine, Tbyama Medical and Pharmaceuticat University, 2630 Sugitani, Tbyama 930-OiP4, Jtu)an. bj21St Century COE Program, 7Q}'ama Mledical and Pharmaceuticat Uhivenylty, 2630 Sagitani, lb.Fama 930-OIV4, mpan. ofKampo Pharmaceutieal Sciences, Nihon Pharmaceutical University, 10281 Komuro, lna, Saitama 362-0...

Sir, We read with great interest the recent article by Floris et al. (2004) on the application of iron oxide particles as MR contrast medium in experimental autoimmune encephalitis (EAE). Infiltration of iron-labelled inflammatory cells was visualized in vivo by MRI. Most notably, local iron accumulation was present at an early clinical stage of disease, i.e. day 11. For assessment of the integ...

Iron is an essential element in the metabolism of all cells. Elevated levels of the metal have been found in the brains of patients of numerous neurodegenerative disorders, including Parkinson's disease (PD). The pathogenesis of PD is largely unknown, although it is thought through studies with experimental models that oxidative stress and dysfunction of brain iron homeostasis, usually a tightl...

Iron is essential for cellular development and maintenance of multiple physiological processes in the central nervous system. The disturbance of its homeostasis leads to abnormal iron deposition in the brain and causes neurotoxicity via generation of free radicals and oxidative stress. Iron toxicity has been established in the pathogenesis of Parkinson's disease; however, its contribution to mu...

Neurodegeneration with brain iron accumulation (NBIA) is a group of genetic diseases characterized by progressive extrapyramidal symptoms and focal iron accumulation in the basal ganglia. β-Propeller protein-associated neurodegeneration (BPAN), also known as static encephalopathy of childhood with neurodegeneration in adulthood or NBIA 5, is an X-linked dominant subtype of NBIA.1 Brain MRI stud...