The hemoglobin-deficit mouse: Cure of the anemia following bone marrow transplantation with normal marrow

The hemoglobin-deficit mouse mutant (hbd) is characterized by a severe microcytic anemia that is inherited in an autosomal-recessive manner. Previous results from our laboratory indicated that normal mice develop anemia if they are transplanted with bone marrow from mutant animals. Furthermore, we demonstrated a delay in erythroid reconstitution from hbd marrow compared to normal marrow. Although these experiments show a defect that is intrinsic to hbd marrow, it is unclear if the hbd phenotype is solely the result of a bone marrow-derived defect. To exclude an environmental influence on hbd anemia, we attempted to cure the defect by transplanting normal marrow into the hbd mouse. We observed that the transplanted animals converted to a normal phenotype. These results indicated that the defect is bone marrow derived. In contrast to the microcytosis mutant whose defective gene is ubiquitiously expressed, our data suggest that the defective gene product is specific to hematopoietic cells.