TABLE 15-1
G lycogen Storage D iseases
Type
Eponym
la
von Gierke’s disease
(hepatorenal
glycogenosis)
lb
Id
II
Pompe’s disease
(generalized
glycogenesis)
III
Forbes’ disease, Cori’s
disease (limit
dextrinosis)
IV
Andersen’s disease
(branching deficiency;
amylopectinosis)
Deficiency
Glucose-
6
-phosphatase in
liver, intestine, and kidney
Glucose-
6
-phosphate
transporter in hepatocyte
microsomal membrane
Phosphate transporter in
hepatocyte microsomal
membrane
Glucose transporter GLUT-7
Lysosomal
a -
1,4-
glucosidase (acid maltase)
Amylo-1,
6
-glucosidase
(debranching enzyme)
( 1,4—> 1,
6
)-transglucosylase
(branching enzyme)
