NDLI: Congenital Nonspherocytic Hemolytic Anemia, Associated with Glutathione Deficiency of the Erythrocytes

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Congenital Nonspherocytic Hemolytic Anemia, Associated with Glutathione Deficiency of the Erythrocytes

Content Provider	Scilit
Author	Prins, H. K. Oort, M. D. M. Loos, J. A. Zürcher, M. D. C. Beckers, T.
Copyright Year	1966
Description	1. A new biochemical defect of erythrocytes is described: glutathione deficiency (reduced glutathione less than 10 per cent of the amount of reduced glutathione in normal erythrocytes). 2. The defect is associated with a clinical picture of congenital nonspherocytic hemolytic anemia which is fairly well compensated. 3. The results of a family study are consistent with an autosomal recessive pattern of inheritance. 4. Labeling with Na2Cr51O4 has a damaging effect on glutathione-deficient erythrocytes. The erythrocyte life span, as estimated by a serological method (Ashby), was markedly shortened (30 days instead of 100-120 days). 5. Red cell destruction could be increased by the administration of primaquine. 6. Secondary to the glutathione deficiency, low glyoxalase activity was observed. The glutathione-reducing capacity, glycolytic activity, and the ATP level of the abnormal red cells were found to be within the normal range. 7. On incubation of the glutathione-deficient erythrocytes in vitro with glycine-C14 and glutamine-C14, no formation of labeled glutathione could be demonstrated.
Related Links	https://ashpublications.org/blood/article-pdf/27/2/145/572670/145.pdf
Ending Page	166
Page Count	22
Starting Page	145
DOI	10.1182/blood.v27.2.145.145
Journal	Blood
Issue Number	2
Volume Number	27
Language	English
Publisher	American Society of Hematology
Publisher Date	1966-02-01
Access Restriction	Open
Subject Keyword	Critical Care Medicine Endocrinology and Metabolism Anemia Erythrocytes Congenital Defect Hemolytic C14 Reduced Glutathione Glutathione Deficiency Journal: Blood (Vol- 44, Issue- 2)
Content Type	Text
Resource Type	Article

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