title: The use of hypotransferrinemic mice in studies of iron biology.
abstract: The hypotransferrinemic (hpx) mouse is a model of inherited transferrin deficiency that originated several decades ago in the BALB/cJ mouse strain. Also known as the hpx mouse, this line is almost completely devoid of transferrin, an abundant serum iron-binding protein. Two of the most prominent phenotypes of the hpx mouse are severe anemia and tissue iron overload. These phenotypes reflect the essential role of transferrin in iron delivery to bone marrow and regulation of iron homeostasis. Over the years, the hpx mouse has been utilized in studies on the role of transferrin, iron and other metals in a variety of organ systems and biological processes. This review summarizes the lessons learned from these studies and suggests possible areas of future exploration using this versatile yet complex mouse model.
authors: Department of Pathology and Laboratory Medicine, Brown University, 70 Ship Street, Providence, RI, 02912, USA.
authors: Julia T
authors: JT
authors: Bu
authors: Thomas B
authors: TB
authors: Bartnikas
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D007501:Iron / Q000378:metabolism; D019190:Iron Overload / Q000378:metabolism / Q000473:pathology; D033862:Iron-Binding Proteins / Q000378:metabolism; D051379:Mice; D014168:Transferrin / Q000378:metabolism*
chemical_list: D033862:Iron-Binding Proteins; D014168:Transferrin; D007501:Iron