Pathogenesis of Hemolytic Anemia in Homozygous Hemoglobin C Disease*
AUTOR(ES)
Charache, Samuel
RESUMO
Hemoglobin C is less soluble than hemoglobin A in red cells, in hemolysates, and in dilute phosphate buffer. Its relative insolubility may be explained by electrostatic interactions between positively charged β6-lysyl groups and negatively charged groups on adjacent molecules. Red cells from patients with homozygous hemoglobin C (CC) disease exhibit aberrant physical properties which suggest that the cells are more rigid than normal erythrocytes. They pass through membrane filters less readily than normal red cells do, and their viscosity is higher than that of normal cells. Differences from normal cells are exaggerated if mean corpuscular hemoglobin concentration (MCHC) is increased, by suspension in hypertonic salt solution. Increased rigidity of CC cells, by accelerating their fragmentation, may be responsible for formation of microspherocytes. These small dense cells are exceptionally rigid, and probably are even more susceptible to fragmentation and sequestration. Rigidity of CC cells can be attributed to a “precrystalline” state of intracellular hemoglobin, in which crystallization does not occur, although the MCHC exceeds the solubility of hemoglobin in hemolysates.
ACESSO AO ARTIGO
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=292930Documentos Relacionados
- Regulation of cation content and cell volume in hemoglobin erythrocytes from patients with homozygous hemoglobin C disease.
- Molecular and cellular pathogenesis of hemoglobin SC disease.
- Magnitude of the fetal hemoglobin response to acute hemolytic anemia in baboons is controlled by genetic factors.
- OCULAR COMPLICATIONS IN SICKLE-CELL HAEMOGLOBIN C DISEASE*
- Studies on the Pathogenesis of Pigment Gallstones in Hemolytic Anemia: DESCRIPTION AND CHARACTERISTICS OF A MOUSE MODEL