Respiratory characteristics of blood from Basenji dogs with classical erythrocyte pyruvate kinase deficiency.
The oxygen affinities of blood from eight Basenji dogs homozygous for classical erythrocyte pyruvate kinase deficiency and four dogs heterozygous for the defect were compared with blood from 14 Labrador retrievers and two normal Basenji dogs. The homozygous dogs showed significant anemia compared to heterozygous and normal dogs (P is less than 0.01). The average blood P50 value (at 38C and plasma pH of 7.40) for both homozygous and heterozygous dogs was significantly higher (P is less than 0.01) than for normal dogs (33.6+/-0.4 and 31.8+/-0.7 vs 30.8+/-0.6 mm Hg). The concentrations of 2,3-DPG in the blood of both heterozygous and homozygous dogs were significantly higher than normal values. Four months after splenectomy P50 values declined to normal in four homozygous Basenji dogs without any change in the degree of anemia or blood 2,3-DPG concentrations. Iron kinetic studies showed a shorter plasma clearance time in a homozygous than in a normal dog with the heterozygote falling midway between. The red cell life span in the normal and heterozygous dogs was approximately 120 days. The 59Fe studies on the homozygous dog indicate markedly different survival characteristics which can be attributed to the existence of three populations of red cells differing in their life spans.
D000818:Animals; D004163:Diphosphoglyceric Acids / Q000097:blood; D004285:Dogs; D004912:Erythrocytes / Q000201:enzymology / Q000378:metabolism*; D006400:Hematocrit; D006454:Hemoglobins / Q000032:analysis; D006579:Heterozygote; D006720:Homozygote; D007501:Iron / Q000097:blood; D010100:Oxygen / Q000097:blood*; D011770:Pyruvate Kinase / Q000172:deficiency*; D013156:Splenectomy
D004163:Diphosphoglyceric Acids; D006454:Hemoglobins; D007501:Iron; D011770:Pyruvate Kinase; D010100:Oxygen