Hereditary defect of intestinal iron transport in mice with sex-linked anemia.

Iron transport by everted duodenal sacs in vitro was studied in mice with sex-linked anemia (gene symbol sla) (an inherited iron deficiency anemia), in normal mice, and in normal mice on iron-deficient and iron supplemented diets. Although the over-all mucosal uptake of iron was the same in sla and normal sacs, transport of iron to the inside of the sac was much decreased in sla. The iron transport defect in sla was emphasized by the fact that genotypically normal mice on an iron-deficient diet demonstrated greatly increased iron transport. Electrophoretic analysis of protein extracted from sla and normal sacs showed only one iron-binding fraction. The sla and normal fractions had the same mobility and corresponded in position to the major band of horse ferritin. It thus appears that the iron deficiency of sla is due to a genetically determined defect in mucosal iron transport and that this defect is not associated with any demonstrable abnormality of a major iron-binding protein.