title: Delayed onset of hemolytic anemia in CBA-Pk-1slc/Pk-1slc mice with a point mutation of the gene encoding red blood cell type pyruvate kinase.
abstract: The Pk-1slc gene encodes a mutant red blood cell (RBC) type pyruvate kinase (PK), and adult CBA-Pk-1slc/Pk-1slc mice show a severe nonspherocytic hemolytic anemia. However, the number of RBCs and the proportion of reticulocytes were comparable between neonatal CBA-Pk-1slc/Pk-1slc mice and control -+/+ mice. Since the age-dependent increase of RBCs was much greater in CBA-+/+ mice than in CBA-Pk-1slc/Pk-1slc mice, significant anemia was observed in the latter mice on day 14 after birth. The increase of RBCs in CBA-+/+ mice was due to the prolongation of their survival time. The half life of RBCs increased in CBA-+/+ mice with ages, but it decreased in CBA-Pk-1slc/Pk-1slc mice. The relatively longer half life of RBCs in neonatal CBA-Pk-1slc/Pk-1slc mice appeared to be due to the delayed switching from M2-type PK that are expressed by undifferentiated erythroid precursor cells to RBC-type PK that are expressed by mature RBCs.
authors.affiliation: Department of Pathology, Osaka University Medical School, Suita, Osaka, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Tsujino
authors.forename: H
authors.initials: H
authors.lastname: Kanno
authors.forename: K
authors.initials: K
authors.lastname: Hashimoto
authors.forename: H
authors.initials: H
authors.lastname: Fujii
authors.forename: T
authors.initials: T
authors.lastname: Jippo
authors.forename: E
authors.initials: E
authors.lastname: Morii
authors.forename: Y M
authors.initials: YM
authors.lastname: Lee
authors.forename: H
authors.initials: H
authors.lastname: Asai
authors.forename: S
authors.initials: S
authors.lastname: Miwa
authors.forename: Y
authors.initials: Y
authors.lastname: Kitamura
mesh_terms: D017668:Age of Onset; D000743:Anemia, Hemolytic / Q000235:genetics*; D000818:Animals; D000831:Animals, Newborn; D000833:Animals, Suckling; D004912:Erythrocytes / Q000201:enzymology*; D004920:Erythropoiesis; D005260:Female; D005838:Genotype; D007527:Isoenzymes / Q000097:blood / Q000172:deficiency / Q000235:genetics*; D008297:Male; D051379:Mice; D008808:Mice, Inbred CBA; D008817:Mice, Mutant Strains; D017354:Point Mutation; D011770:Pyruvate Kinase / Q000097:blood / Q000172:deficiency / Q000235:genetics*
chemical_list: D007527:Isoenzymes; D011770:Pyruvate Kinase