Association of hemoglobin H disease with Hb J-Iran (beta 77 His----Asp): impact on subunit assembly.

Abstract

A young Iranian female was found to be heterozygous for hemoglobin (Hb) J-Iran (beta 77 His----Asp) in combination with Hb H disease. The proportion of Hb J in the patient's hemolysate was surprisingly high: 65% Hb J, 30% Hb A. Thus, the interaction of a negatively charged beta subunit variant of Hb with alpha-thalassemia leads to a marked increase in the relative amount of the variant Hb within red cells. This observation provides further support for an electrostatic model of Hb subunit assembly.

• Authors: S Rahbar, H F Bunn
• Journal: Blood (Blood) Vol. 70 Issue 6 Pg. 1790-1 (Dec 1987) ISSN: 0006-4971 [Print] United States
• PMID: 3676514 (Publication Type: Journal Article)

Chemical References

• Hemoglobins, Abnormal
• Macromolecular Substances
• Hemoglobin J
• hemoglobin J Iran
• Globins

Topics

• Globins (genetics)
• Hemoglobin J
• Hemoglobins, Abnormal
• Humans
• Macromolecular Substances
• Structure-Activity Relationship
• Thalassemia (genetics)