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Polyamines and globin binding in sickle cell disease

C L Natta, A A Motyczka, L T Kremzner

The American Journal of Pediatric Hematology/Oncology

|January 1, 1982

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Sickle cell anemia patients have higher spermine levels in red blood cells. This polyamine may contribute to red blood cell membrane defects in sickle cell disease.

Area of Science:

Biochemistry
Hematology
Molecular Biology

Background:

Sickle cell anemia is a genetic blood disorder characterized by abnormal hemoglobin.
Red blood cell membrane abnormalities are a hallmark of sickle cell disorders.
The role of polyamines in red blood cell physiology is not fully understood.

Purpose of the Study:

To investigate the levels of spermine in erythrocytes from sickle cell anemia patients.
To explore the potential role of spermine in the membrane abnormalities associated with sickle cell disease.

Main Methods:

Quantification of spermine levels in erythrocytes from sickle cell anemia patients and healthy controls.
Analysis of the binding of beta s globin to red cell stroma.

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Main Results:

Erythrocytes from sickle cell anemia patients exhibited five to ten times higher spermine concentrations compared to controls.
Spermine may mediate the preferential binding of beta s globin to the red cell membrane.

Conclusions:

Elevated spermine levels in sickle cell erythrocytes are a significant finding.
Spermine is implicated as a potential factor contributing to the membrane pathophysiology in sickle cell disorders.