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Differential pathways in oxy and deoxy HbC aggregation/crystallization.

R E Hirsch1, R E Samuel, N A Fataliev

  • 1Department of Medicine, Division of Hematology, Albert Einstein College of Medicine and Montefiore Hospital, Comprehensive Sickle Cell Center, Bronx, New York 10461, USA. rhirsch@aecom.yu.edu

Proteins
|November 28, 2000
PubMed
Summary
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Hemoglobin C (HbC) crystallization differs between oxygenated and deoxygenated states. Oxy HbC favors tetragonal crystal formation, while deoxy HbC exhibits diverse aggregates and hexagonal crystals, suggesting ligand-dependent conformational effects.

Area of Science:

  • Biophysics
  • Hematology
  • Molecular Biology

Background:

  • Hemoglobin C (HbC) disease is characterized by intraerythrocytic hemoglobin crystals.
  • The formation of HbC crystals in vivo is primarily observed in the oxygenated state, but the underlying reasons are not fully understood.

Purpose of the Study:

  • To investigate and compare the in vitro crystallization behavior of oxygenated (oxy) and deoxygenated (deoxy) Hemoglobin C (HbC).
  • To explore the influence of ligand-binding state on HbC aggregation and crystal morphology.

Main Methods:

  • HbC crystallization was induced in vitro using concentrated potassium phosphate buffer.
  • Differential interference contrast microscopy was employed to visualize and compare crystal formation in oxy and deoxy HbC.

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Main Results:

  • Oxy HbC formed amorphous aggregates and subsequent tetragonal crystals, with some macro-ribbon structures.
  • Deoxy HbC also formed aggregates and macro-ribbons but displayed greater morphologic diversity, including unbranched fibers, with infrequent crystal formation.
  • Deoxy HbC formed flat, hexagonal crystals, distinct from the tetragonal crystals of oxy HbC.

Conclusions:

  • The crystallization process of HbC is dependent on its ligand-binding conformation (oxy R-state vs. deoxy T-state).
  • The oxygenated state thermodynamically favors pathways leading to tetragonal crystal formation, while the deoxygenated state allows for multiple aggregation pathways, not all resulting in crystals.