Reaction: Defective SI does not hydrolyze maltotriose
- in pathway: Intestinal saccharidase deficiencies
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 maltotriose to maltose and glucose, based on the experimentally demonstrated failure of these SI mutant proteins to hydrolyze maltose (e.g., Sander et al. 2005) and the broad substrate specificity of the normal enzyme (Sim et al. 2010).
Reaction - small molecule participants:
H2O [extracellular region]
maltotriose [extracellular region]
Reactome.org reaction link: R-HSA-5659899
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Reaction input - small molecules:
water
alpha-maltotriose
Reaction output - small molecules:
Reactome.org link: R-HSA-5659899