Role Of Macro And Micronutrients In The Management Of Glycogen Storage Disease Manifestations – A Preclinical Study

Abstract

ABSTRACT Background Glycogen storage disease type 1 (GSD type 1) is an inherited metabolic disease resulted due to the impaired glucose-6-phosphatase enzyme complex, which is important in glycogen metabolism. The enzyme complex glucose-6-phosphatase (G6Pase), is encoded by G6PC1 gene, and the glucose-6 phosphate translocase or transporter subunit (G6PT), which is encoded by the SLC37A4 gene. The major manifestations of GSD type 1 are hypoglycemia, excessive glycogen accumulation, hepatomegaly, hyperuricemia, hyperlactatemia. This study investigates the induction and management of GSD I symptoms. It is known that metformin may affect the levels of lactic acid, and that chlorogenic acid (CGA), which is prevalent in green coffee and present in various kinds of fruits, inhibits the enzyme glucose-6-phosphatase complex and micronutrients which inhibits the glycogenesis process were employed for the management of hepatic glycogen accumulation, which is responsible for further other manifestations as mentioned above. Objectives: To establish a chemically induced GSD model, which could facilitate to investigate the most common GSD type 1 manifestations and to investigate the influence of dietary regimen consisting of macronutrients and micronutrients in experimentally induced GSD manifestations.