Novel Approaches in Glycogen Storage Disease Type I Management: Harnessing the Potential of Micronutrients and Macro Molecules

Background: Genetic metabolic illnesses called Glycogen Storage Disease type I (GSD I) are caused due to abnormalities in the glucose-6-phosphatase enzyme complex involved in the metabolism of glycogen. Objectives: The current study explores the induction and management of GSD I manifestations. Materials and Methods: Chlorogenic Acid (CGA), a hydroxycinnamic acid found in various fruits and abundant in green coffee, can inhibit the enzyme glucose-6- phosphatase complex and metformin can alter the lactic acid levels. Results: The in silico and in vivo approach unveiled, that β-carotene showed the highest binding affinity with glucokinase, forming 7 hydrogen bonds. Riboflavin exhibited the highest binding affinity with protein phosphatase 1, forming 4 hydrogen bonds. Vitamin C interacted at the active site of the control ligand. Vitamin E also displayed significant binding affinity with glucokinase. Overall, these bioactive micro molecules show promising interactions with their respective target proteins. In vivo results revealed the significant impact of Macronutrients and Micronutrients, particularly when combining Cassava starch with the micronutrients. This combination showed notable improvements in various parameters, including liver glycogen levels, liver weight, fasting blood glucose levels, and liver histology. The findings suggest the potential of this synergistic approach in enhancing liver function and glucose regulation. To conclude this work, it was evident that the combination of Macro and Micronutrients revealed distinct advantages. UCCS and Cassava starch effectively maintained normoglycemia due to their sustained glucose release from complex carbohydrates. Remarkably, Cassava starch, both alone and combined with micronutrients, outperformed UCCS in promoting better outcomes. This highlights the potential for comprehensive research on Cassava starch's effects in GSD subjects, exploring its impact on other manifestations such as lactic acidosis, hypertriglyceridemia, and hyperuricemia. Conclusion: These findings offer promising avenues for further investigation and potential therapeutic applications.