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Metabolic Disorders of the Erythrocyte
JAMA. 1970;211(5):820-821.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings. |
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The erythrocyte, once considered to be an inert bag of hemoglobin, is now known to contain complex metabolic machinery which is essential in maintaining the normal functions of this cell. The metabolic structure of the erythrocyte may be divided into three compartments: the glycolytic pathway, which supplies the energy necessary for maintaining ionic gradients and the discoid form; the hexose monophosphate shunt-glutathione system, which protects the cell from oxidative injury; and the methemoglobin reductase pathway, which helps maintain hemoglobin iron in the functional ferrous form. Twenty-five or more enzymatic steps are involved in these pathways; many of the enzymes have been isolated and characterized and some of the control mechanisms have been delineated. Specific erythrocyte enzyme deficiencies are now often recognized as the pathogenic factors in the miscellaneous group of hereditary nonspherocytic hemolytic anemias (HNSHA).
At least 13 enzymatic abnormalities of the red cell have been described, usually resulting in
. . . [Full Text PDF of this Article]
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