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 (2,3-diphosphoglycerate
This shift on left side is balanced by increased production of 2,3-diphosphoglycerate
by red blood cells after prominent increase elevation; in vivo P50 stays approximately the same as at the sea level, and the mechanism in turn produces a compensatory rightward shift in the curve [13, 14].
Beta thalassemia syndromes are disorders which are inherited and are characterized by deficiency in the production of beta globin chains resulting in ineffective erythropoiesis complicated by lack of affinity of circulating haemoglobin F to 2,3-diphosphoglycerate
. As a consequence of this, repeated blood transfusions are needed to maintain life, which in turn results in excessive iron being deposited in various organs resulting in early fatalities.
However, it is the result of the fact that the cell energy pathways, such as clinical ATP and 2,3-diphosphoglycerate
, are affected in severe cases.
First, the pika obtained oxygen effectively by larger pulmonary alveoli superficial and higher capillary density (Wang et al., 2008a), thin walled pulmonary arterioles and blunted hypoxic pulmonary vasoconstriction (Ge et al., 1998), an increase in erythrocyte count (Wang et al., 2008b), reduction in the mean corpuscular volume (Ye et al., 1994), changes in hemoglobin (Hb) (He et al., 1994) and 2,3-diphosphoglycerate
concentrations (Ge et al., 1998), and an increase in the oxygen affinity to Hb (He et al., 1994).
Important dysfunction includes reduced oxygen delivery resulting from progressive decrease in 2,3-diphosphoglycerate
(2,3-DPG) concentrations , reduced PRBC-dependent vasodilation  (for review see [14, 15]), decreased cell membrane deformability [16,17] which potentially obstructs capillary flow, consumption of clotting factors, and activation of intravascular coagulation through PRBC derived microvesicles .
In addition, autologous blood has low acid content and normal K+ concentration, relatively higher 2,3-diphosphoglycerate
levels and provides better cell vitality, preventing complications such as hyperkalemia.
This might be explained by the fact that hypophosphataemia causes deficiency in the intermediary compounds for energy production such as adenosine triphosphate and 2,3-diphosphoglycerate
and alterations in energy metabolism, which may lead to respiratory muscle weakness and consequent worsening of respiratory insufficiency.
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).
Effects of multibuffer supplementation on acid-base balance and 2,3-diphosphoglycerate
following repetitive anaerobic exercise.
There is an elevation of 2,3-diphosphoglycerate
(2,3-DPG) that is stimulated by a rise in intracellular pH.