Meta-Biol

• Pathways

Mutations that disrupt the catalytic activity or strongly interfere with proper folding, glycosylation and transport of SI (sucrase-isomaltase) are inferred to block the cleavage of sucrose (Suc) to glucose and fructose, based on the experimentally demonstrated failure of these SI mutant proteins to hydrolyze maltose (e.g., Sander et al. 2006) and the broad substrate specificity of the normal enzyme (Sim et al. 2010).