Table 3.

Erythrocyte Enzyme Levels of Patient N.S.

Enzyme or Intermediate Level Normal Range PatientMother Brother
Hexokinase  0.67 to 1.79  3.26 
Glucose phosphate isomerase  25.45 to 51.60  40.50 
Phosphofructokinase (PFK)  3.02 to 6.38  2.18  
Aldolase 1.01 to 3.29  6.10  
Triose phosphate isomerase 805 to 1971  1463  
Glyceraldehyde phosphate dehydrogenase 119.5 to 258.4  190.6  
Diphosphoglycerate mutase 1.21 to 5.27  5.29  
Phosphoglycerate kinase 175.9 to 260.6  223.4  
Monophosphoglycerate mutase 13.40 to 24.10  32.56  
Enolase  2.91 to 6.00  5.49 
Pyruvate kinase (PK)  7.32 to 16.0  22.7  
Lactate dehydrogenase  60.4 to 214.0  170.9  
Glucose-6-phosphate dehydrogenase  9.3 to 14.2  21.8  
6-Phosphogluconate dehydrogenase  6.75 to 10.70  15.85  
Glutathione reductase 6.62 to 8.83  7.62  
Glutathione peroxidase 21.40 to 40.30  25.77  
GOT  2.19 to 5.20  15.71 
Adenylate kinase  87.7 to 241.0  192.0  
GST 1.45 to 4.15  0.83  
Pyrimidine 5′ nucleotidase (mU/g Hb) 100.5 to 176.1  262.7  
Adenosine deaminase 0.70 to 1.60  0.92  
GSH  4.50 to 8.70  0.21 5.14  5.31  
γ-Glutamylcysteine synthetase 0.88 to 1.98  0.14  0.42  0.70  
Glutathione synthetase  0.24 to 0.53  0.40 
Enzyme or Intermediate Level Normal Range PatientMother Brother
Hexokinase  0.67 to 1.79  3.26 
Glucose phosphate isomerase  25.45 to 51.60  40.50 
Phosphofructokinase (PFK)  3.02 to 6.38  2.18  
Aldolase 1.01 to 3.29  6.10  
Triose phosphate isomerase 805 to 1971  1463  
Glyceraldehyde phosphate dehydrogenase 119.5 to 258.4  190.6  
Diphosphoglycerate mutase 1.21 to 5.27  5.29  
Phosphoglycerate kinase 175.9 to 260.6  223.4  
Monophosphoglycerate mutase 13.40 to 24.10  32.56  
Enolase  2.91 to 6.00  5.49 
Pyruvate kinase (PK)  7.32 to 16.0  22.7  
Lactate dehydrogenase  60.4 to 214.0  170.9  
Glucose-6-phosphate dehydrogenase  9.3 to 14.2  21.8  
6-Phosphogluconate dehydrogenase  6.75 to 10.70  15.85  
Glutathione reductase 6.62 to 8.83  7.62  
Glutathione peroxidase 21.40 to 40.30  25.77  
GOT  2.19 to 5.20  15.71 
Adenylate kinase  87.7 to 241.0  192.0  
GST 1.45 to 4.15  0.83  
Pyrimidine 5′ nucleotidase (mU/g Hb) 100.5 to 176.1  262.7  
Adenosine deaminase 0.70 to 1.60  0.92  
GSH  4.50 to 8.70  0.21 5.14  5.31  
γ-Glutamylcysteine synthetase 0.88 to 1.98  0.14  0.42  0.70  
Glutathione synthetase  0.24 to 0.53  0.40 

Activity of enzymes is presented as international units per gram of Hb. GSH levels are millimoles per liter.

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