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Fetal compensation of the hemolytic anemia in mice homozygous for the
normoblastosis (nb) mutation
LL Peters, CS Birkenmeier and JE Barker
Jackson Laboratory, Bar Harbor, ME 04609.
The mouse autosomal recessive mutation nb causes a deficiency of erythroid
ankyrin and generates a life-threatening hemolytic anemia in adult mice;
however, at birth, nb/nb mice appear to be robust and show no pallor. In
our study, the time of disease onset was sought by comparison of nb/nb and
+/? mice both in utero and postnatally. Erythroid ankyrin messenger RNA
(mRNA) is expressed in fetal erythroid progenitors from normal mice, but is
reduced to 10% of normal levels in mutant fetuses. Despite the deficiency
of erythroid ankyrin mRNA, 16 and 18 day nb/nb fetuses have normal levels
of red blood cells (RBCs) and the RBCs are morphologically normal by
scanning electron microscopy. The earliest signs of any clinical anomaly
are an increase in the number of circulating reticulocytes and the
deposition of minor amounts of iron just before birth in the 18 day fetal
nb/nb liver, suggesting that RBCs are being destroyed. Within 24 hours
after birth, nb/nb neonates have a slight but significant decrease of their
RBC counts. During the next 5 days, the nb/nb RBC counts decrease markedly,
the reticulocyte counts assume the mutant adult levels of 60%, the
erythrocytes become microcytic and fragmented, and iron deposits accumulate
in the liver. The rapid onset of clinical disease postnatally, coupled with
our findings that the erythroid ankyrin gene is transcribed in fetal
erythroid cell precursors from normal mice, suggest that mechanisms exist
in the nb/nb fetus to compensate for the erythroid ankyrin deficiency.
Volume 80,
Issue 8,
pp. 2122-2127,
10/15/1992
Copyright © 1992 by The American Society of Hematology
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