Kl. Simon-stoos et Ml. Bloom, The hemoglobin-deficit mouse: Cure of the anemia following bone marrow transplantation with normal marrow, EXP HEMATOL, 27(1), 1999, pp. 162-168
The hemoglobin-deficit mouse mutant (hbd) is characterized by a severe micr
ocytic anemia that is inherited in an autosomal-recessive manner, Previous
results from our laboratory indicated that normal mice develop anemia if th
ey are transplanted with bone marrow from mutant animals. Furthermore, we d
emonstrated 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 hbn a
nemia, we attempted to cure the defect by transplanting normal marrow into
the hbd mouse. We observed that the transplanted animals converted to a nor
mal phenotype, These results indicated that the defect is bone marrow deriv
ed, In contrast to the microcytosis mutant whose defective gene is ubiquiti
ously expressed, our data suggest that the defective gene product is specif
ic to hematopoietic cells. (C) 1999 International Society for Experimental
Hematology. Published by Elsevier Science Inc.