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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap

Data

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Human metabolism

Reactome's human metabolism drawn as one network. Each molecule has one home node; widely shared molecules also appear as dashed copies in other territories, linked from their details. .

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Metabolite; its structure appears as you zoom in
Reaction, arrow toward the product
Cofactors and co-substrates are written beside the reaction when you zoom in
Dashed ring: a copy of a widely shared molecule or cofactor in another territory or pathway
Compartment transfer of the molecule it is linked to
Binding, release or complex event; its proteins are named in its details
Outline: reactions mostly in the mitochondrion

Arrows follow Reactome's curated reaction direction.

GAA hydrolyzes lysosomal glycogen

glycogen-GYG1 dimer + 2 H2O → poly((1,4)-alpha-glucosyl)GYG1 dimer + 2 Glc

Reactome: R-HSA-9036727
Compartment: lysosomal lumen
Rhea: None recorded

Inputs

glycogen-GYG1 dimerR-HSA-9036726, lysosomal lumen
2 H2OCHEBI:15377, H2O, lysosomal lumen

Outputs

poly((1,4)-alpha-glucosyl)GYG1 dimerR-HSA-5357533, lysosomal lumen
2 GlcCHEBI:4167, C6H12O6, lysosomal lumen

Catalysed by

alpha-glucosidase activity of GAA (70, 76 kDa) [lysosomal lumen]EC 3.2.1.20

Literature

Simultaneous absence of alpha-1,4-glucosidase and alpha-1,6-glucosidase activities (pH 4) in tissues of children with type II glycogen storage disease Biochemistry, 1970
Primary structure and processing of lysosomal alpha-glucosidase; homology with the intestinal sucrase-isomaltase complex EMBO J., 1988
Specific functions of lysosomal proteases in endocytic and autophagic pathways Biochim. Biophys. Acta, 2012
Structural and functional changes of lysosomal acid alpha-glucosidase during intracellular transport and maturation J. Biol. Chem., 1993