PURPOSE It is well known that patients with Wilson's disease (WD) suffer copper metabolism disorder. However, recent studies point to an additional iron metabolism disorder in WD patients. The purpose of our study was to examine susceptibility-weighted imaging (SWI) manifestations of WD in the brains of WD patients. METHODS A total of 33 patients with WD and 18 normal controls underwent conve...

The WD-repeat domain is a highly conserved recognition module in eukaryotes involved in diverse cellular processes. It is still not well understood how the bottom of a WD-repeat domain recognizes its binding partners. The WD-repeat-containing protein EED is one component of the PRC2 complex that possesses histone methyltransferase activity required for gene repression. Here we report the crysta...

Wilson disease (WD) is an autosomal recessive disease of copper metabolism due to mutations in the ATP7B gene on chromosome 131. Patients may show a disabling movement disorder characterized by extrapyramidal signs with dystonia and choreoathetosis, associated with psychiatric signs and liver involvement, usually occurring during the first decades of life. Copper chelation by D-penicillamine re...

wilson's disease is a rare but treatable condition with variable clinical presentations. its diagnosis depends on a combination of clinical and laboratory findings. we evaluated the clinical and laboratory findings in children with wilson's disease (wd). twenty -seven children (4-14 years, 59.2 % male, 40.7% female) with confirmed wd were evaluated between 1994 and 2003 at imam khomei...

The study aimed to verify the effects of vitamin D3 (cholecalciferol) supplementation on thebody fat composition and plasmatic oxidative stress obese rats. 21 days old maleWistar rats were acclimatized in sectorial animal breed until they 30 old(CEUA nº 5866200720). Then obesity induction was initiated using cafeteria diet(Western Diet; WD). animals divided into 2 groups: CTL, fed a normal diet...

Wilson's disease (WD) is an autosomal recessive inherited disorder of copper metabolism that results in the accumulation of copper in the body and primarily in the liver, brain, and cornea. Copper is a toxic metal and might be associated with cancer induction. Most malignancies associated with WD are hepatocellular carcinoma and cholangiocarcinoma. Other intra-abdominal malignancies have been o...

INTRODUCTION Wilson's disease is an inherited disorder caused by a gene located on chromosome 13, which involved copper transportation across cell membranes. The disease can cause a reduced incorporation of copper into ceruloplasmin resulting in accumulation of this metal in the liver, central nervous system, kidneys and other organs. The objective is to define the frequencies of psychiatric di...

There is evidence that increased release of corticotropin-releasing factor (CRF) in the central nucleus of the amygdala (CeA) contributes to stress responsivity during cocaine withdrawal (WD). Recent studies suggest that tissue plasminogen activator (tPA) in the CeA is a downstream effector protein for CRF after acute "binge" cocaine administration. The purpose of this study was to determine if...

BACKGROUND AND PURPOSE Wilson disease (WD) is rare but one of the few metabolic disorders that can possibly benefit from effective available treatments. The literature regarding proton MR spectroscopy (MRS) in WD is scarce and controversial. The purpose of this study was to determine the brain metabolic changes due to WD by using MRS. To our knowledge, this is the first time that MRS was perfor...

Calcium channel blockers slow the progression of atherosclerosis. The purpose of the present experiments was to examine the action of lacidipine in a condition that accelerates the development of atherosclerosis in order to test the hypothesis that the protective action of lacidipine in atherosclerosis is unrelated to the reduction of blood pressure. Male ApoE-deficient mice (6 weeks old) were ...