The inherited mutations in PKR genes cause a deficit in cellular energy within the red blood cell, as evidenced by lower PK enzyme activity, a decline in adenosine triphosphate levels and a build-up of upstream metabolites, including 2,3-DPG
In neonates clinical and haematological picture is more severe due to increase level of Hb F as it increases the level of 2,3-DPG
which destabilizes spectrin-actin interaction thus exacerbating the defect.
The biochemical changes include the alternation in the concentration of adenosine triphosphate (ATP), 2,3-DPG
, NADH, and the intracellular/extracellular ion distribution.
A number of mechanisms have been proposed to provide benefit following SP ingestion which include an enhanced 2,3-diphosphoglycerate (2,3-DPG
) concentration, which allows for greater unloading of oxygen to the peripheral tissues/muscle (Buck et al., 2013; Benesch and Benesch, 1969; Duhm, 1971) and an improved buffering capacity due to increased hydrogen phosphate concentration, which could buffer hydrogen ions produced during intense exercise (Kreider, 1999).
There is an elevation of 2,3-diphosphoglycerate (2,3-DPG
) that is stimulated by a rise in intracellular pH.
To draw a standard curve, we determined the concentrations of the intercellular adhesion molecule-1 (ICAM-1), advanced glycation end-product receptor (RAGE), tumor necrosis factor-[alpha] (TNF-[alpha]), and 2,3-DPG
by ELISA according to the manufacturer's instructions (antibodies were purchased from R&D Systems, USA).
Fetal hyperglycemia, fetal hyperinsulinemia, as well as decreased 2,3-DPG
levels causing an increased affinity of maternal hemoglobin for oxygen can lead to lactic acidosis and hypoxia in the fetus (2),(5).
But stored blood loses a chemical called 2,3-DPG
(for 2,3 diphosphoglycerate) that facilitates the unloading of oxygen from hemoglobin molecules on red blood cells.
The second factor that results in decreased oxygen dissociation may be even more pervasive, and that is a decrease in the molecule 2,3,di-phoshoglycerate (2,3-DPG
Alkaline CPD and the preservation of RBC 2,3-DPG
. Transfusion, v.42, p.747-752, 2002.
La afinidad de la Hb por el [O.sub.2] esta influida por una serie de variables que incluyen la concentracion de protones, el C[O.sub.2], la temperatura y el 2,3-difosfoglicerato (2,3-DPG
These findings suggest that the absence of proline [beta]5 slightly affects the spatial positions of Val at NA1 and His at NA2 and indirectly affects the off-loading of 2,3-DPG
as observed in Hb Warwickshire.