Glycoside hydrolase family 31 (GH31)

Glycoside hydrolase family 31 (GH31) represents a clinically significant group of enzymes responsible for the hydrolysis of alpha-glycosidic linkages in complex sugars and glycoconjugates (CAZy Database, 2024). In humans, the family is most notably represented by acid alpha-glucosidase (GAA), sucrase-isomaltase (SI), and maltase-glucoamylase (MGAM) (UniProtKB - P10253, P13717). GAA is a lysosomal enzyme essential for glycogen degradation; its absence results in Pompe disease (Glycogen Storage Disease Type II), characterized by progressive muscle autophagic failure and cardiomyopathy (NINDS, 2023). SI and MGAM are brush-border enzymes that facilitate the final steps of starch digestion in the small intestine. Pharmacological modulation of GH31 enzymes is an established strategy: alpha-glucosidase inhibitors (e.g., acarbose) are used to treat type 2 diabetes by slowing glucose release from dietary carbohydrates, while recombinant GAA (e.g., alglucosidase alfa) serves as life-saving enzyme replacement therapy for Pompe disease (DrugBank Online, 2024). Recent drug development also explores pharmacological chaperones to stabilize mutant GH31 enzymes.

Other names

GH family 31Alpha-glucosidase familyFamily 31 glycosyl hydrolaseAcid alpha-glucosidaseSucrase-isomaltaseMaltase-glucoamylase

The mechanism of action involves the competitive inhibition of intestinal alpha-glucosidase enzymes to delay carbohydrate digestion and glucose absorption, or the replacement of deficient lysosomal alpha-glucosidase to enable glycogen hydrolysis (DrugBank Online, 2024).