Exploring the role of hepcidin, an antimicrobial and iron regulatory peptide, in increased iron absorption in beta-thalassemia.
To develop new treatments for beta-thalassemia, it is essential to identify the genes involved in the relevant pathophysiological processes. Iron metabolism in thalassemia mice being investigated, focusing on the expression of a gene called hepcidin (Hamp), which is expressed in the liver and whose product (Hamp) is secreted into the bloodstream. In mice, iron overload leads to overexpression of Hamp, while Hamp-knockout mice suffer from hemochromatosis. The aim of this study is to investigate Hamp in the mouse model of beta-thalassemia and to address the potential gene transfer of Hamp to prevent abnormal iron absorption.
Breda
Laura
L
Weill Medical College of Cornell University, Department of Pediatrics, Division of Hematology-Oncology, Children's Blood Foundation Laboratories, New York, New York 10021, USA.
Gardenghi
Sara
S
Guy
Ella
E
Rachmilewitz
Eliezer A
EA
Weizer-Stern
Orly
O
Adamsky
Konstantin
K
Amariglio
Ninette
N
Rechavi
Gideon
G
Giardina
Patricia J
PJ
Grady
Robert W
RW
Rivella
Stefano
S
D000818:Animals; D023181:Antimicrobial Cationic Peptides / Q000096:biosynthesis / Q000235:genetics / Q000502:physiology*; D006528:Carcinoma, Hepatocellular / Q000473:pathology; D002460:Cell Line / Q000378:metabolism; D045744:Cell Line, Tumor; D004195:Disease Models, Animal; D005347:Fibroblasts / Q000378:metabolism; D005786:Gene Expression Regulation; D005822:Genetic Vectors / Q000627:therapeutic use; D006432:Hemochromatosis / Q000235:genetics* / Q000378:metabolism; D022781:Hepatocytes / Q000378:metabolism; D064451:Hepcidins; D006801:Humans; D007408:Intestinal Absorption / Q000502:physiology*; D007501:Iron / Q000493:pharmacokinetics*; D019190:Iron Overload / Q000209:etiology* / Q000235:genetics / Q000378:metabolism; D016086:Lentivirus / Q000235:genetics; D008099:Liver / Q000378:metabolism; D008113:Liver Neoplasms / Q000473:pathology; D051379:Mice; D018345:Mice, Knockout; D041681:NIH 3T3 Cells; D014161:Transduction, Genetic; D017086:beta-Thalassemia / Q000378:metabolism / Q000628:therapy
D023181:Antimicrobial Cationic Peptides; C578702:HAMP protein, human; C579599:Hamp protein, mouse; D064451:Hepcidins; D007501:Iron