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Blood, 15 December 2005, Vol. 106, No. 13, pp. 4034-4042.
Prepublished online as a Blood First Edition Paper on July 28, 2005; DOI 10.1182/blood-2005-04-1622.


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REVIEW ARTICLES

The energy-less red blood cell is lost: erythrocyte enzyme abnormalities of glycolysis

Richard van Wijk, and Wouter W. van Solinge

From the Department of Laboratory Medicine, University Medical Center Utrecht, Utrecht, The Netherlands.

The red blood cell depends solely on the anaerobic conversion of glucose by the Embden-Meyerhof pathway for the generation and storage of high-energy phosphates, which is necessary for the maintenance of a number of vital functions. Many red blood cell enzymopathies have been described that disturb the erythrocyte's integrity, shorten its cellular survival, and result in hemolytic anemia. By far the majority of these enzymopathies are hereditary in nature. In this review, we summarize the current knowledge regarding the genetic, biochemical, and structural features of clinically relevant red blood cell enzymopathies involved in the Embden-Meyerhof pathway and the Rapoport-Luebering shunt.


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