Autosomal dominant hypophosphatemic rickets (ADHR) is unique among the disorders involving Fibroblast growth factor 23 (FGF23) because individuals with R176Q/W and R179Q/W mutations in the FGF23 (176)RXXR(179)/S(180) proteolytic cleavage motif can cycle from unaffected status to delayed onset of disease. This onset may occur in physiological states associated with iron deficiency, including pub...

Xanthine oxidoreductase (XOR) may produce reactive oxygen species and play a role in ischemia-reperfusion injury. Because tissue iron levels increase after ischemia, and because XOR contains functionally critical iron-sulfur clusters, we studied the effects of intracellular iron on XOR expression. Ferric ammonium citrate and FeSO(4) elevated intracellular iron levels and increased XOR activity ...

Genetic disorders of iron metabolism and chronic inflammation often evoke local iron accumulation. In Friedreich ataxia, decreased iron-sulphur cluster and heme formation leads to mitochondrial iron accumulation and ensuing oxidative damage that primarily affects sensory neurons, the myocardium, and endocrine glands. We assessed the possibility of reducing brain iron accumulation in Friedreich ...

Plants are excellent source of natural antioxidants which have beneficial effect on health. The present study was designed to assess the antioxidant and reactive oxygen species (ROS) scavenging activities of 70% methanolic extract of Clerodendrum viscosum. The extracts were examined for the scavenging activity of hydroxyl radical, nitric oxide, singlet oxygen, hypochlorous acid, superoxide, per...

iron chelation therapy is used to reduce iron overload development due to its deposition in various organs such as liver and heart after regular transfusion. in this review, different iron chelators implicated in treatment of iron overload in various clinical conditions have been evaluated using more up-to-date studies focusing on these therapeutic agents. deferoxamine, deferiprone and deferasi...

Heart failure secondary to iron overload is the main cause of death in patients with beta-thalassemia major. Combination therapy with deferoxamine and deferiprone has been shown to be more effective than either drug used alone in patients with beta-thalassemia major and symptomatic cardiomyopathy. Although monitoring the response to chelation therapy is usually carried out by indirect measureme...

TREATMENT OF LEAD TOXICITY with chelation was first reported with EDTA in the early 1950s. Apparent success in reducing metastatic calcium deposits led Clarke et al in 1956 to treat angina patients with EDTA, and others to use chelation for various forms of atherosclerotic disease. Chelation therapy evolved to constitute infusions of vitamins and disodium EDTA, a drug that binds divalent and so...

Iron is an essential cellular nutrient, being a critical cofactor of several proteins involved in cell growth and replication. Compared with normal cells, neoplastic cells have been shown to require a greater amount of iron, thus laying the basis for the promising anticancer activity of iron chelators. In this work, we evaluated the effects of molecules with iron chelation activity on neuroblas...

Regular red cell transfusion therapy ameliorates disease-related morbidity and can be lifesaving in patients with various hematological disorders. Transfusion therapy, however, causes progressive iron loading, which, if untreated, results in endocrinopathies, cardiac arrhythmias and congestive heart failure, hepatic fibrosis, and premature death. Iron chelation therapy is used to prevent iron l...

BACKGROUND Beta-thalassemia major (β-TM) patients need blood transfusions, which result in iron deposition. To regulate chelation therapy, iron load has to be measured. With MRI, the amount of signal loss and T2* decay time shortening are used for iron quantification. OBJECTIVES The aim was to measure adrenal iron load with T2* relaxometry using MRI, and to compare it with liver and cardiac i...