• intercrosses of heterozygotes yields only 16% homozygotes, less than mendelian prediction, indicating in utero loss

• the white blood cell count is normal (J:138919)

• spleens are normal in weight and iron content (J:138919)

• red blood cells are osmotically fragile, shperocytic, hypochromic, microcytic, and have a lower maximum deformability index with ektacytometry showing a loss of membrane surface area

• more pronounced in males than females

• in both males and females

• in both males and females

• in males

• red blood cells have diminished membrane surface area relative to that in wildtype controls

• indicative of a compensatory increase in the rate of erythropoiesis

• found in both males and females

• platelet counts are elevated significantly in males and to a lesser extent in females

• mean platelet volume is significantly lower in females and lower in males to a level that did not reach statistical significance

• platelet shape and bleed time are normal (J:138919)

• plasma BUN and electrolyte concentrations are normal (J:138919)

• approximately half of homozygotes develop hydrocephaly, and on average this phenotype is overtly visible by 8 weeks of age

• progressive dilation of the lateral and third ventricles with the cerebral aqueduct open resulting in accumulation of fluid within the fourth ventricle

• homozygotes are smaller than normal at birth and throughout life

• average weight of only approximately 10g at 5 weeks of age is much less than littermate controls