The role of raw starches in the treatment of type I glycogenosis
JB Sidbury, YT Chen, CR Roe - Archives of internal medicine, 1986 - jamanetwork.com
The glycogenoses are a group of heritable disorders characterized by an abnormality in
glycogen metabolism. The first to be described and enzymatically defined, type I glycogen
storage disease (GSD), was characterized by an absence or deficiency of glucose-6-
phosphatase. 1 Indeed, this was the first inborn error of metabolism in which the absent or
deficient enzyme was demonstrated, 52 years after Sir Archibald Garrod's prediction in 1902
that inborn errors would be found to be due to an absence of a single enzyme. 2 Although …
glycogen metabolism. The first to be described and enzymatically defined, type I glycogen
storage disease (GSD), was characterized by an absence or deficiency of glucose-6-
phosphatase. 1 Indeed, this was the first inborn error of metabolism in which the absent or
deficient enzyme was demonstrated, 52 years after Sir Archibald Garrod's prediction in 1902
that inborn errors would be found to be due to an absence of a single enzyme. 2 Although …