نتایج جستجو برای: hemochromatosis

تعداد نتایج: 2753  

Journal: :Blood 2002
Richard S Ajioka Joanne E Levy Nancy C Andrews James P Kushner

Hereditary hemochromatosis is most commonly caused by homozygosity for a point mutation (C282Y) in the human hemochromatosis gene (HFE). The mechanism by which HFE regulates iron absorption is not known, but the C282Y mutation results in loss of cell surface expression of the human hemachromatosis protein (HFE) and hyperabsorption of iron by the duodenal enterocyte. Mice homozygous for a deleti...

Journal: :Human molecular genetics 2014
Isabelle Callebaut Rozenn Joubrel Serge Pissard Caroline Kannengiesser Victoria Gérolami Cécile Ged Estelle Cadet François Cartault Chandran Ka Isabelle Gourlaouen Lénaick Gourhant Claire Oudin Michel Goossens Bernard Grandchamp Hubert De Verneuil Jacques Rochette Claude Férec Gérald Le Gac

Hemochromatosis type 4 is a rare form of primary iron overload transmitted as an autosomal dominant trait caused by mutations in the gene encoding the iron transport protein ferroportin 1 (SLC40A1). SLC40A1 mutations fall into two functional categories (loss- versus gain-of-function) underlying two distinct clinical entities (hemochromatosis type 4A versus type 4B). However, the vast majority o...

Journal: :Postgraduate medical journal 1965
I D Heath

INSULIN resistance in association with primary haemochromatosis has been reported on several occasions previously (Allen and Constam, 1929; Root, 1929; Wood and Fitzhugh, 1929; Engel, 1934; Eskind, Franklin and Lowell, 1953; Colwell and Weiger, 1956; Darnaud, Denard, Moreau, Voisin and Pujol, 1958; Uhry, Marcel and Cohen, 1958; Justin-Besancon, Pequignot, Deuil, Etienne, Magdelaine and Jullien,...

Journal: :Clinical chemistry 1998
M Vernet J Y Le Gall

concentration of proteins in Hemoce, whereas the others detected various concentrations in the solution. The Hemoce dilutions and the results obtained after processing these dilutions on different analyzers are shown in Fig. 1. Because Hemoce is itself a mixture of polypeptides, it is inaccurate to say that Hemoce positively interferes with some urine protein methods. Nevertheless, when determi...

2014
Bulent Acikyol Ross M. Graham Debbie Trinder Michael J. House John K. Olynyk Rodney J. Scott Elizabeth A. Milward Daniel M. Johnstone

(2013) Brain transcriptome perturbations in the transferrin receptor 2 mutant mouse support the case for brain changes in iron loading disorders, including effects relating to long-term depression and long-term potentiation. Neuroscience, 235. pp. 119-128. NOTICE: this is the author's version of a work that was accepted for publication in Neuroscience. Changes resulting from the publishing proc...

2008
Eugénia Cruz Chris Whittington Samuel H Krikler Cláudia Mascarenhas Rosa Lacerda Jorge Vieira Ana Martinelli Jill Waalen

Title: A new 500kb haplotype associated with high CD8+ T-lymphocyte numbers predicts a less severe expression of hereditary hemochromatosis

Journal: :iranian red crescent medical journal 0
sina gerayli department of biology, faculty of sciences, ferdowsi university of mashhad, mashhad, ir iran; department of biology, western university, london, ontario, n6a 5b7, canada alireza pasdar department of modern sciences and technologies, faculty of medicine, mashhad university of medical sciences, mashhad, ir iran; division of applied medicine, medical school, university of aberdeen, foresterhill, aberdeen, ab25 2zd, uk mohammad taghi shakeri department of biostatistics, public health school, mashhad university of medical sciences, mashhad, ir iran samaneh sepahi targeted drug delivery research center, school of pharmacy, mashhad university of medical sciences, mashhad, ir iran seyed mousalreza hoseini department of gastroenterology and hepatology, ghaem hospital, faculty of medicine, mashhad university of medical sciences, mashhad, ir iran mitra ahadi department of gastroenterology and hepatology, ghaem hospital, faculty of medicine, mashhad university of medical sciences, mashhad, ir iran

conclusions polymorphism in the hemochromatosis gene may confer some degree of risk for hcv infection, and individuals carrying the h and c alleles may be susceptible to this disease; however, a larger sample of hcv patients and healthy individuals may be necessary to further illustrate the role of these polymorphisms in hcv. patients and methods hfe gene polymorphisms were examined in a total ...

Journal: :فصلنامه علوم پزشکی دانشگاه آزاد اسلامی واحد پزشکی تهران 0
آگاه محمدرضا agah mr research center for gastroenterology and liver diseases, shaheed beheshti university of medical sciences, tehran, iranمرکز تحقیقات بیماری های گوارش و کبد، دانشگاه علوم پزشکی شهید بهشتی ظفرقندی مریم zafarghandi m research center for gastroenterology and liver diseases, shaheed beheshti university of medical sciences, tehran, iranمرکز تحقیقات بیماری های گوارش و کبد، دانشگاه علوم پزشکی شهید بهشتی مطهری زهرا motahari z research center for gastroenterology and liver diseases, shaheed beheshti university of medical sciences, tehran, iranمرکز تحقیقات بیماری های گوارش و کبد، دانشگاه علوم پزشکی شهید بهشتی جزایری هانیه السادات jazaeri h research center for gastroenterology and liver diseases, shaheed beheshti university of medical sciences, tehran, iranمرکز تحقیقات بیماری های گوارش و کبد، دانشگاه علوم پزشکی شهید بهشتی زالی محمدرضا hajibeigi b research center of iranian blood transfusion organization, tehran, iranمرکز تحقیقات سازمان انتقال خون ایران حاجی بیگی بشیر attarchy z research center of iranian blood transfusion organization, tehran, iranمرکز تحقیقات سازمان انتقال خون ایران عطارچی زهره

background: there are no data on the frequency and biochemical expression of the hemochromatosis associated mutations, c282y and h63d, in iranian adult population. this is the first study among iranians that may advocate a screening program. materials and methods: we investigated the frequency of the c282y/h63d hfe gene mutations in a group of 1029 randomly selected iranian blood donors as well...

Journal: :Haematologica 2000
S Parkkila A K Parkkila A Waheed R S Britton X Y Zhou R E Fleming S Tomatsu B R Bacon W S Sly

BACKGROUND AND OBJECTIVE Most patients with hereditary hemochromatosis are homozygous for a Cys282AETyr mutation in the HFE gene. This mutation has been shown to impair the association of the HFE gene product with b(2)-microglobulin and to prevent its cell surface presentation in transfected COS-7 and 293 cells. This study was performed to examine the expression of HFE protein in epithelial cel...

Journal: :Blood 2007
Ivana De Domenico Michael B Vaughn Donghoon Yoon James P Kushner Diane M Ward Jerry Kaplan

The term hemochromatosis represents a group of inherited disorders leading to iron overload. Mutations in HFE, HJV, and TfR2 cause autosomal-recessive forms of hemochromatosis. Mutations in ferroportin, however, result in dominantly inherited iron overload. Some mutations (H32R and N174I) in ferroportin lead to macrophage iron loading, while others (NI44H) lead to hepatocyte iron loading. Expre...

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