Red cell membrane remodeling in sickle cell anemia. Sequestration of membrane lipids and proteins in Heinz bodies.

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Red cell membrane remodeling in sickle cell anemia. Sequestration of membrane lipids and proteins in Heinz bodies.

In red cells from patients with sickle cell anemia, hemoglobin S denatures and forms Heinz bodies. Binding of Heinz bodies to the inner surface of the sickle cell membrane promotes clustering and colocalization of the membrane protein band 3, outer surface-bound autologous IgG and, to some extent, the membrane proteins glycophorin and ankyrin. Loss of transbilayer lipid asymmetry is also found ...

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Hepatic sequestration in sickle cell anemia.

INTRODUCTION Sickle cell anemia is a chronic debilitating disease affecting a significant portion of patients of African American origin. These patients present to the physicians with myriad of life threatening complications like acute chest syndrome, septic shock, decompensated congestive heart failure secondary to severe pulmonary hypertension, stroke, and multi-organ failure. However, there ...

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Side Effects of Hydroxyurea in Patients with Sickle Cell Anemia

Background: Hemoglobin S arises is the result of a point mutation (A-T) in the sixth codon on the -globin gene on chromosome 11 causing sickle cell anemia. The presence of fetal hemoglobin in infancy plays a relatively protective role for vaso-occlusive symptoms that are the major contributor for the morbidity and mortality among patients with sickle cell anemia. hydroxyurea, an s-phase-specif...

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Reduced erythrocyte membrane protein methylation in sickle cell anemia.

The methylation of erythrocyte membrane components in sickle cell anemia has been studied and found to differ considerably from that of normal erythrocytes. When sickle erythrocytes were incubated under physiological conditions (pH = 7.4, 37 degrees C) in the presence of L-[methyl-3H]methionine or S-adenosyl-L-[methyl-3H] methionine, a 50% decrease in the protein-carboxyl methylation was observ...

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ژورنال

عنوان ژورنال: Journal of Clinical Investigation

سال: 1996

ISSN: 0021-9738

DOI: 10.1172/jci118402