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Denatured hemoglobin in sickle erythrocytes
The Journal of Clinical Investigation
|April 1, 1977
Summary
Sickle cell disease (Hb SS) red blood cells contain significantly more denatured hemoglobin compared to normal red blood cells (Hb AA). This suggests sickle hemoglobin
Area of Science:
- Hematology
- Biochemistry
- Cell Biology
Background:
- Sickle cell disease (Hb SS) is characterized by abnormal hemoglobin S.
- Inclusion bodies are frequently observed in red blood cells of Hb SS patients.
- The nature of these inclusion bodies requires further investigation.
Purpose of the Study:
- To investigate the composition and origin of inclusion bodies in sickle cell disease red blood cells.
- To quantify the amount of denatured hemoglobin associated with red blood cell membranes in Hb SS.
Main Methods:
- Preparation of red cell ghosts free of oxyhemoglobin.
- Spectrophotometric analysis of ghost suspensions.
- Heme extraction and quantification.
- Analysis of membrane-associated heme after sodium dodecyl sulfate treatment.
Main Results:
- Red cell ghosts from Hb SS patients showed absorption spectra typical of hemichromes, indicating denatured hemoglobin.
- Hb SS red cells contained approximately five times more denatured hemoglobin than Hb AA cells.
- Quantified denatured hemoglobin was significantly higher in Hb SS patients (0.158%) compared to controls (0.030%).
Conclusions:
- The increased denatured hemoglobin in Hb SS red cells is likely linked to the inherent instability of sickle oxyhemoglobin.
- Denatured hemoglobin may contribute to the formation of inclusion bodies observed in sickle cell disease.
- Further research is needed to elucidate the precise mechanisms of hemoglobin denaturation and inclusion body formation in sickle cell disease.
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