12-09-2009, 11:48 AM
A sample of glycogen from a newborn with liver disease is incubated with inorganic phosphate, normal glycogen phosphorylase, and debranching enzyme. The ratio of glucose-1-phosphate (G-1-P) to glucose formed in the reaction is abnormally high. The patient is most likely deficient in which of the following?
A. alpha-1,6-glucosidase
B.(alpha -1,4 ”alpha-1 ,4) glucan transferase
C.(alpha-1,4 ”alpha-1,6) glucan transferase
D. Glycogen phosphorylase
E. Glycogen phosphorylase kinase
A. alpha-1,6-glucosidase
B.(alpha -1,4 ”alpha-1 ,4) glucan transferase
C.(alpha-1,4 ”alpha-1,6) glucan transferase
D. Glycogen phosphorylase
E. Glycogen phosphorylase kinase