Diphosphoglycerate and Phenotypic Severity of Cell Disease

Gezeery, Amina R.; Abdulaban, Fatima S.; Al-Musa, Mahasin A.

doi:10.21608/jhiph.2004.187331

Diphosphoglycerate and Phenotypic Severity of Cell Disease

Document Type : Original Article

Authors

¹ Human Genetics Department, Medical Research Institute, Alexandria University, Egypt

² Biochemistry Department, Faculty of Science, King Saud University

³ Hematology Department, Riyadh and Alkarj Hospital, Riyadh, KSA

10.21608/jhiph.2004.187331

Abstract

Sickle cell disease is a hereditary blood disorder characterized by chronic manifestations of progressive organ failure. To control a disease of this type, it is important to study the factors which affect the course of the disease. Diphosphoglycerate [2,3 DPG] is the most common metabolite generated by the glycolysis. It is a potent modifier for hemoglobin function. To clarify the role of 2,3 DPG in suckling, its concentration was measured in 15 normal health individuals and in 12 sickle cell patients at steady state and during crisis. Hematological indices and partial oxygen tension [PO2] were also determined for sicklers and controls. Sickle cell patients during crisis were found to have a significant higher levels of 2,3 DPG [9.17+5.36 mold/L] [p < 0.05], and significantly low PO2 [8.41 +1.82 Kpa, p < 0.01] than controls [8.54 + 0.72 mmol/L for 2,3 DPG and 12.44 + 0.7 Kpa for PO4]. The increase in 2,3 DPG concentration during crisis may be due to its increased production via glycolysis, which in turn increased as a result of decreased oxygen tension during crisis. The increased 2,3 DPG concentration increases and stabilized the deoxygenated HbS leading to its precipitation, sickling. And hemolysis of red blood cells. In conclusion, the factors which can increase 2,3 DPG or decrease PO4 may lead to the sickling crisis and increase the phenotypic severity of the disease.

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