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Biochemistry Glycogen a127d562

Hypoglycemia is more severe in type 1 Glycogen storage disease as compared to type 6 Glycogen storage disease because :

A
No gluconeogenesis in type 1 disease
B
No gluconeogenesis in type 6 disease
C
Both
D
Type 1 disease affects muscles and liver both
High-Yield Explanation
Table: Comparison of Type I and Type VI glycogen storage disease Type I (Von Gierke's disease) Type VI (Her's disease) Deficiency of glucose-6-Phosphatase(this enzyme is common to bothglycogenolysis and gluconeogenesis) Deficiency of GlycogenPhosphorylase No Gluconeogenesis Gluconeogenesis not affected No glycogenolysis No Glycogenolysis Severe hypoglycaemia Mild Hypoglycemia Ketosis occurs Ketosis does not occur As is shown in table, gluconeogenesis is not affected in Type VI so no severe hypoglycemia in type 6 as compared to Type I where gluconeogenesis is affected.

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