Abstract |
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.
|
Authors | C Brugnara, A S Kopin, H F Bunn, D C Tosteson |
Journal | Transactions of the Association of American Physicians
(Trans Assoc Am Physicians)
Vol. 97
Pg. 104-12
( 1984)
ISSN: 0066-9458 [Print] United States |
PMID | 6535334
(Publication Type: Comparative Study, Journal Article)
|
Chemical References |
- Electrolytes
- Hemoglobin C
- Hemoglobin A
|
Topics |
- Acid-Base Equilibrium
- Electrolytes
(blood)
- Erythrocytes
(metabolism)
- Hemoglobin A
(genetics)
- Hemoglobin C
(genetics)
- Hemoglobin C Disease
(blood, metabolism)
- Humans
|