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  • Title: [Mechanisms of congenital erythrocyte enzyme deficiencies associated with hemolytic anemia].
    Author: Boivin P, Kahn A.
    Journal: Ann Biol Clin (Paris); 1976; 34(1):53-62. PubMed ID: 135522.
    Abstract:
    The search for a mechanism for red cell enzyme deficiency associated with congenital hemolytic anemia, requires one to determine the kinetic and thermodynamic properties of the enzyme reaction and study the physico-chemical and immunological characteristics of the protein which supports enzyme activity. The technique of iso-electric focalisation and the use of specific anti-enzyme antibodies, is the reason for recent progress in the understanding of the mechanism of these deficiencies. Examples of application of these techniques are given in relation to glucose-6-dehydrogenase, pyruvate kinase, glucose phosphate isomerase, phosphofructokinase and phosphoglycerate kinase of deficiencies showing the multiplicity of the molecular mechanisms.
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