Tag Priya S. Kishnani

Defects in Metabolism of Carbohydrates

Carbohydrate synthesis and degradation provide the energy required for most metabolic processes. The important carbohydrates include 3 monosaccharides—glucose, galactose, and fructose—and a polysaccharide, glycogen. Fig. 105.1 shows the relevant biochemical pathways of these carbohydrates. Glucose is the principal substrate of…

Glycogen Storage Diseases

Definition Glycogen storage diseases are a group of disorders resulting from inherited defects in the biochemical pathways of glycogen metabolism. Based on organ involvement, glycogen storage diseases are broadly categorized into two groups: hepatic glycogen storage diseases and muscle glycogen…