Table 1
Enzymatic activities
Phosphorylase b kinase (nmol/min/g Hb)
Phosphorylase (nmol/min/mg)
Debranching enzyme (nmol glucose/h/mg prot)
Erythrocytes
Leucocytes
Leucocytes
Patient
89.1
2.2
0.3
Mother
53.4
1.7
6.8
Control
63.9
2.7
4.1