Anemia in β-thalassemia patients targets hepatic hepcidin transcript levels independently of iron metabolism genes controlling hepcidin expression

被引:26
作者
Camberlein, Emilie [1 ]
Zanninelli, Giuliana [2 ]
Detivaud, Lenaick [1 ]
Lizzi, Anna Rita [2 ]
Sorrentino, Francesco [2 ]
Vacquer, Stefania [4 ]
Troadec, Marie-Berengere [1 ]
Angelucci, Emanuele [3 ]
Abgueguen, Emmanuelle [1 ]
Loreal, Olivier [1 ]
Cianciulli, Paolo [2 ]
Lai, Maria Eliana [4 ]
Brissot, Pierre [1 ]
机构
[1] Univ Hosp Pontchaillou, Inserm U522, IFR 140, Rennes, France
[2] Day Hosp Talassemie, Osped San Eugenio, Rome, Italy
[3] Osped Oncol, Div Ematol, Cagliari, Italy
[4] Univ Cagliari, Osped Regionale Microcitemie, Ctr Talassem Adulti, Dipartimento Sci Med, Cagliari, Italy
关键词
anemia; beta-thalassemia; hepcidin; gene expression; iron overload;
D O I
10.3324/haematol.11656
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Thalassemia associates anemia and iron overload, two opposite stimuli regulating hepcidin gene expression. We characterized hepatic hepcidin expression in 10 thalassemia major and 13 thalassemia intermedia patients. Hepcidin mRNA levels were decreased in the thalassemia intermedia group which presented both lower hemoglobin and higher plasma soluble transferrin receptor levels. There was no relationship between hepcidin mRNA levels and those of genes controlling iron metabolism, including HFE, hemojuvelin, transferrin receptor-2 and ferroportin. These results underline the role of erythropoietic activity on hepcidin decrease in thalassemic patients and suggest that mRNA modulations of other studied genes do not have a significant impact.
引用
收藏
页码:111 / 115
页数:5
相关论文
共 20 条
[1]   Understanding iron homeostasis through genetic analysis of hemochromatosis and related disorders [J].
Camaschella, C .
BLOOD, 2005, 106 (12) :3710-3717
[2]   Ineffective erythropoiesis in β-thalassemia is characterized by increased iron absorption mediated by down-regulation of hepcidin and up-regulation of ferroportin [J].
Gardenghi, Sara ;
Marongiu, Maria F. ;
Ramos, Pedro ;
Guy, Ella ;
Breda, Laura ;
Chadburn, Amy ;
Liu, YiFang ;
Amariglio, Ninette ;
Rechavi, Gideon ;
Rachmilewitz, Eliezer A. ;
Breuer, William ;
Cabantchik, Z. Ioav ;
Wrighting, Diedra M. ;
Andrews, Nancy C. ;
de Sousa, Maria ;
Giardina, Patricia J. ;
Grady, Robert W. ;
Rivella, Stefano .
BLOOD, 2007, 109 (11) :5027-5035
[3]   Expression of hepcidin in hereditary hemochromatosis: evidence for a regulation in response to the serum transferrin saturation and to non-transferrin-bound iron [J].
Gehrke, SG ;
Kulaksiz, H ;
Herrmann, T ;
Riedel, HD ;
Bents, K ;
Veltkamp, C ;
Stremmel, W .
BLOOD, 2003, 102 (01) :371-376
[4]  
HUEBERS HA, 1990, BLOOD, V75, P102
[5]   Diferric transferrin regulates transferrin receptor 2 protein stability [J].
Johnson, MB ;
Enns, CA .
BLOOD, 2004, 104 (13) :4287-4293
[6]  
Kattamis A, 2006, HAEMATOLOGICA, V91, P809
[7]   Urinary hepcidin in congenital chronic anemias [J].
Kearney, Susan L. ;
Nemeth, Elizabeta ;
Neufeld, Ellis J. ;
Thapa, Dharma ;
Ganz, Tomas ;
Weinstein, David A. ;
Cunningham, Melody J. .
PEDIATRIC BLOOD & CANCER, 2007, 48 (01) :57-63
[8]   The role of the iron responsive element in the control of ferroportin1/IREG1/MTP1 gene expression [J].
Lymboussaki, A ;
Pignatti, E ;
Montosi, G ;
Garuti, C ;
Haile, DJ ;
Pietrangelo, A .
JOURNAL OF HEPATOLOGY, 2003, 39 (05) :710-715
[9]   Hepcidin, a putative mediator of anemia of inflammation, is a type II acute-phase protein [J].
Nemeth, E ;
Valore, EV ;
Territo, M ;
Schiller, G ;
Lichtenstein, A ;
Ganz, T .
BLOOD, 2003, 101 (07) :2461-2463
[10]   The gene encoding the iron regulatory peptide hepcidin is regulated by anemia, hypoxia, and inflammation [J].
Nicolas, G ;
Chauvet, C ;
Viatte, L ;
Danan, JL ;
Bigard, X ;
Devaux, I ;
Beaumont, C ;
Kahn, A ;
Vaulont, S .
JOURNAL OF CLINICAL INVESTIGATION, 2002, 110 (07) :1037-1044