Table 1.

Content of γ-glutamyl cycle enzymes and products in erythrocytes and leukocytes of 2 GCS-deficient patients

Erythrocytes
GCS Act.*γ-GC†GS Act.*GSH†
1:2  0.412  < 0.01 6.9  0.079  
3:10  < 0.1  < 0.01  4.3  0.132 
Control range  22.7-27.5  0.54-1.49  3.6-8.1 7.5-10.7  
 (n = 6)  (n = 2)  (n = 13) (n = 9) 
Erythrocytes
GCS Act.*γ-GC†GS Act.*GSH†
1:2  0.412  < 0.01 6.9  0.079  
3:10  < 0.1  < 0.01  4.3  0.132 
Control range  22.7-27.5  0.54-1.49  3.6-8.1 7.5-10.7  
 (n = 6)  (n = 2)  (n = 13) (n = 9) 
Leukocytes
GCS Act.‡GSH1-153
1:2  4.79  3.37  
Controls (n = 2)  380-1146 30.6-64.1 
Leukocytes
GCS Act.‡GSH1-153
1:2  4.79  3.37  
Controls (n = 2)  380-1146 30.6-64.1 
*

pkatal/mg Hb.

†

μmol/g Hb.

‡

nkatal/mg protein.

F1-153

μmol/mg protein.

GCS indicates γ-glutamylcysteine synthetase; γ-GC, γ-glutamylcysteine; GS, glutathione synthetase; GSH, glutathione.

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