Electrolyte composition and equilibrium in hemoglobin CC red blood cells.
The red cells of two patients with homozygous hemoglobin C disease (CC) were found to have reduced cation and water content when compared to the red cells of two normal subjects that contained only hemoglobin A (AA). The reduction in cation content was of such a magnitude that the intracellular chloride and hydroxyl (and proton) concentrations were within normal limits despite a measured reduction in the concentration of impermeant negative anions in CC as compared with AA cells of 40 mEq/kg of dry cell solids. The osmotic coefficient of hemoglobin C in CC cells was found to be similar to that observed for hemoglobin A in AA cells. We found no evidence for increased amounts of bound or osmotically abnormal water in CC cells. The reduction in cell cation content in CC cells is mainly due to a reduction in cell K content. The reduced K content is probably related to an increased ouabain- and furosemide-insensitive pathway for K transport in CC cells.
D000136:Acid-Base Equilibrium; D004573:Electrolytes / Q000097:blood*; D004912:Erythrocytes / Q000378:metabolism*; D006441:Hemoglobin A / Q000235:genetics; D006444:Hemoglobin C / Q000235:genetics; D006445:Hemoglobin C Disease / Q000097:blood* / Q000378:metabolism; D006801:Humans
D004573:Electrolytes; D006444:Hemoglobin C; D006441:Hemoglobin A