scholarly journals A Series of New Screening Procedures for Pyruvate Kinase Deficiency, Glucose-6-Phosphate Dehydrogenase Deficiency, and Glutathione Reductase Deficiency

Blood ◽  
1966 ◽  
Vol 28 (6) ◽  
pp. 942-942
Author(s):  
ERNEST BEUTLER ◽  
Agnes Halasz
Keyword(s):  
Glutathione Reductase ◽  
Pyruvate Kinase ◽  
Dehydrogenase Deficiency ◽  
Pyruvate Kinase Deficiency ◽  
Reductase Deficiency ◽  
Screening Procedures ◽  
Glutathione Reductase Deficiency ◽  
Glucose 6 Phosphate Dehydrogenase

Abstract BEUTLER, E.: A SERIES OF NEW SCREENING PROCEDURES FOR PYRUVATE KINASE DEFICIENCY, GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY, AND GLUTATHIONE REDUCTASE DEFICIENCY. Blood 28:553-562, 1966. In the October 1966 issue of Blood, on page 557 the concentration for GSSG was erroneously given as .003 M. The line under B. Reaction Mixture should read: GSSG, .033M 0.1 ml.

scholarly journals A Series of New Screening Procedures for Pyruvate Kinase Deficiency, Glucose-6-Phosphate Dehydrogenase Deficiency, and Glutathione Reductase Deficiency

Blood ◽  
1966 ◽  
Vol 28 (4) ◽  
pp. 553-562 ◽  
Author(s):  
ERNEST BEUTLER ◽  
Agnes Halasz
Keyword(s):  
Glutathione Reductase ◽  
Pyruvate Kinase ◽  
Dehydrogenase Deficiency ◽  
Triosephosphate Isomerase ◽  
General Procedure ◽  
Red Cell ◽  
Pyruvate Kinase Deficiency ◽  
Reductase Deficiency ◽  
Glutathione Reductase Deficiency ◽  
Glucose 6 Phosphate Dehydrogenase

Abstract A new type of screening procedure for the detection of enzymatic defects of the red cell has been described. The blood or red cell sample is added to the reaction mixture. After a suitable period of incubation a drop of the mixture is spotted on filter paper, permitted to dry, and examined for fluorescence under UV light. In this way the oxidation of reduction of pyridine nucleotides is readily evaluated. Reaction mixtures for the detection of glucose-6-phosphate dehydrogenase deficiency, pyruvate kinase deficiency, and glutathione reductase deficiency are described. The same general procedure should be readily adaptable to the detection of other enzymatic deficiencies of red cells, such as phosphogluconate dehydrogenase deficiency or triosephosphate isomerase deficiency.

scholarly journals Isoniazid acetylating phenotype in patients with paracoccidioidomycosis and its relationship with serum sulfadoxin levels, glucose-6-phosphate dehydrogenase and glutathione reductase activities

1991 ◽  
Vol 24 (2) ◽  
pp. 111-114 ◽  
Author(s):  
Benedito Barraviera ◽  
Paulo Câmara Marques Pereira ◽  
Jussara Marcondes Machado ◽  
Maria Julia de Souza ◽  
Carlos Roberto G. Lima ◽  
...  
Keyword(s):  
Glutathione Reductase ◽  
Reductase Activity ◽  
Serum Levels ◽  
Slow Acetylators ◽  
Slow Acetylator ◽  
A Value ◽  
Acti Vity ◽  
Glutathione Reductase Activity ◽  
Glutathione Reductase Deficiency ◽  
Glucose 6 Phosphate Dehydrogenase

The authors evaluated the isoniazid acetylating phenotype and measured hematocrit, hemoglobin, glucose-6-phosphate dehydrogenase and glutathione reductase activities plus serum sulfadoxin levels in 39 patients with paracoccidioidomycosis (33 males and 6 females) aged 17 to 58 years. Twenty one (53.84%) of the patients presented a slow acetylatingphenotype and 18(46.16%) a fast acetylating phenotype. Glucose-6-phosphate- dehydrogenase (G6PD) acti vity was decreased in 5(23.80%) slow acetylators and in 4(22.22%) fast acetylators. Glutathione reductase activity was decreased in 14 (66.66%) slow acetylators and in 12 (66.66%) fast acetylators. Serum levels of free and total sulfadoxin Were higher in slow acetylator (p < 0.02). Analysis of the resultspermitted us to conclude that serum sulfadoxin levels are related to the acetylatorphenotype. Furthermore, sulfadoxin levels were always above 50 µg/ml, a value considered therapeutic. Glutathione reductase deficiency observed in 66% of patients may be related to the intestinal malabsorption of nutrients, among them riboflavin, a FAD precursor vitamin, inpatients with paracoceidioidomycosis.

scholarly journals Protection of phagocytic leukocytes by endogenous glutathione: studies in a family with glutathione reductase deficiency

Blood ◽  
1979 ◽  
Vol 53 (5) ◽  
pp. 851-866 ◽  
Author(s):  
D Roos ◽  
RS Weening ◽  
AA Voetman ◽  
ML van Schaik ◽  
AA Bot ◽  
...  
Keyword(s):  
Glutathione Reductase ◽  
Reductase Deficiency ◽  
Glutathione Reductase Deficiency

scholarly journals Platelet Dysfunction with Glutathione Reductase Deficiency After 1,3-Bis(2 Chloroethyl)-l-Nitrosourea

1977 ◽  
Author(s):  
R. McKenna ◽  
T. Ahmad ◽  
H. Frischer
Keyword(s):  
Glutathione Reductase ◽  
Platelet Dysfunction ◽  
Dose Response Relationship ◽  
Control Activity ◽  
Platelet Factor ◽  
Antitumor Chemotherapy ◽  
Glutathione Reductase Deficiency ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Induced Aggregation

We have previously shown that patients receiving antitumor chemotherapy with 1,3-bis-(2 chloroethyl)-1-nitrosourea (BCNU) rapidly acquire a severe generalized glutathione reductase (GSSG-R) deficiency. We now report that when platelets were exposed in vitro to BCNU (30 minutes, 37°, at 10-3 M, six separate studies), the resulting GSSG-R deficiency was associated with marked impairment of platelet aggregation in response to ADP (1 μM and 3 μM), to epinephrine and to collagen (all p’s< 0. 001). Platelet factor 3 availability was also markedly reduced (p<0.001); prothrombin consumption and glass adhesiveness were unaffected. In additional experiments to evaluate the dose response relationship, epinephrine induced aggregation was abnormal at 5 × 10-6 M BCNU, ADP (1 μM) induced aggregation was abnormal at 10-5 M BCNU, while ADP (3 μM) and collagen aggregation became abnormal at 5 × 10-5 M BCNU. GSSG-R deficiency (less than 11% of control activity), without glucose-6-phosphate dehydrogenase and 6-phosphogluconic dehydrogenase deficiencies, preceded all platelet abnormalities. We have demonstrated that a specific platelet GSSG-R deficiency after BCNU precedes the development of severe platelet dysfunction.

Glutathione Reductase Deficiency Alters Lung Development in Newborn Mice

2010 ◽  
Vol 49 ◽  
pp. S51
Author(s):  
Joseph Luchsinger ◽  
Lynette K Rogers ◽  
Morgan L Locy ◽  
Cynthia L Hill ◽  
Markus Velten ◽  
...  
Keyword(s):  
Glutathione Reductase ◽  
Lung Development ◽  
Newborn Mice ◽  
Reductase Deficiency ◽  
Glutathione Reductase Deficiency
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