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Target intelligence / Profile preview
Hemoglobin S (HbS)
- Target
- HbS
- Molecular classification
- Hemoglobin, Globin, Tetrameric protein
01
Overview
Hemoglobin S (HbS) is an abnormal variant of normal adult hemoglobin (hemoglobin A) resulting from a single amino acid substitution: valine replaces glutamic acid at the sixth position of the beta (β) globin chain. This mutation causes deoxygenated HbS molecules to polymerize under low oxygen conditions, distorting red blood cells into a rigid "sickle" or crescent shape. This leads to vaso-occlusion, pain crises, organ damage, and anemia, which are hallmarks of sickle cell disease.
- Other names
- Hgb SSickle cell hemoglobin
02
Mechanism of action
Hydroxyurea increases fetal hemoglobin (HbF) production, which reduces the proportion of HbS. Voxelotor inhibits HbS polymerization. Crizanlizumab reduces vaso-occlusion by binding to P-selectin.
03
Biological functions
Oxygen transportRed blood cell function
04
Disease associations
Sickle cell diseaseAnemiaVaso-occlusionChronic painOrgan damageIncreased infection risk
05
Safety considerations
Drug resistanceAdverse drug reactions (e.g., myelosuppression with hydroxyurea)Vaso-occlusive crises despite treatmentRisk of infection
06
Interacting drugs
Hydroxyurea
3 more in the full profile.
07
Biomarkers
HbS levelHbF levelReticulocyte countHemoglobin level
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