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heart diseases

G6PD Deficiency And Anemia - Glucose 6-phosphate Dehydrogenase Deficiency

g6pd deficiency and anemia


G6PD deficiency may cause anemia. Red blood cells are vulnerable to injury by oxidizing agents. Our body has specialized mechanisms that prevent such injuries to happen. Reduced glutathione (represented as GSH) is one of the most important anti-oxidant present in the body. Various enzymes are needed in the formation of glutathione. Any defficiency of these enzymes decreases the formation of reduced glutathione and renders the RBC's vulnerable to injury by exogenous or endogenous oxidizing agents.
Glucose 6-phosphate dehydrogenase is the important enzyme that is commonly missing or abnormal.
Deficiency of this enzyme leads to decreased formation of reduced glutathione (GSH). In the absence of GSH RBCs become very vulnerable to the injury by oxidizing agents.
Hydrogen peroxide is the most common oxidizing agent that is formed in the body. In the absence of reduced glutathione, it damages the RBC membrane, haemoglobin chains andcauses precipitation of Haemoglobin chains (called Heinz bodies)  and that ultimately results in easy destruction of RBCs when they pass through the narrow splenic vascular bed which causes anemia.
Note:
Thats how reduced glutathione removes hydrogen peroxide.
H2O2 + GSH                       2 H2O + GSSG(oxidized glutathione)

Who are affected More, Males or Females?:

Gene of G6PD is present on X chromosome. Male gets only one X chromosome, and if the X chromosome that he gets is abnormal he will get the disease.
On the other hand, Females have two X chromosomes. For severe disease both of the X chromosomes should be abnormal, but this is less common.
Those females who have one Normal and other abnormal X chromosome have two populations of RBCs, One population has normal RBCs with G6PD present and other has abnormal RBCs with G6PD absent. Such females don't show symptoms except in special cases.

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