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Title: Heme biosynthesis and drug metabolism in mice with hereditary hemolytic anemia

Journal Article · · J. Biol. Chem.; (United States)
OSTI ID:6296061

Mice homozygous for the nb (normoblastosis) gene have severe hemolytic anemia characterized by increased catabolism were measured in the tissues of homozygous (nb/nb), heterozygous (+/nb), and control (+/+) mice generated on the same genetic background. The functional capacity of the microsomal hemeprotein, cytochrome P-450, was also determined in the livers of these animals. Mice homozygous for the nb gene defect had a marked increase in protoporphyrin content, delta-aminolevulinic acid (ALA)-dehydratase, and uroporphyrinogen I (URO)-synthase activities in erythrocytes. Lesser increases were observed in liver and spleen of nb/nb mice. The homozygous mice also had a marked increase in microsomal heme oxygenase activity in the liver, kidney, and spleen compared to normal controls. The increase in heme oxygenase activity is attributable to a higher specific activity per mg of microsomal protein in the case of the liver and the kidney and to the marked organamegaly in the case of the spleen.

OSTI ID:
6296061
Journal Information:
J. Biol. Chem.; (United States), Vol. 254:3
Country of Publication:
United States
Language:
English