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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap

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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.

bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3

GM2A:GM2 + H2O → GalNAc + GM3 + GM2A(32-193)

Reactome: R-HSA-1605595
Compartment: lysosomal lumen
Rhea: None recorded

Inputs

GM2A:GM2R-HSA-1605600, lysosomal lumen
H2OCHEBI:15377, H2O, lysosomal lumen

Outputs

GalNAcCHEBI:28037, C8H15NO6, lysosomal lumen
GM3CHEBI:60065, C42H72N2O21R, lysosomal lumen
GM2A(32-193)R-HSA-1605614, lysosomal lumen

Catalysed by

beta-N-acetylhexosaminidase activity of bHEXA [lysosomal lumen]EC 3.2.1.52

Literature

Oligosaccharide structure and amino acid sequence of the major glycopeptides of mature human beta-hexosaminidase Biochemistry, 1988
Molecular basis of an adult form of beta-hexosaminidase B deficiency with motor neuron disease Biochem Biophys Res Commun, 1991
A point mutation in the coding sequence of the beta-hexosaminidase alpha gene results in defective processing of the enzyme protein in an unusual GM2-gangliosidosis variant J Neurochem, 1988
Purification and characterization of an activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A Hoppe Seylers Z Physiol Chem, 1979
Crystallographic structure of human beta-hexosaminidase A: interpretation of Tay-Sachs mutations and loss of GM2 ganglioside hydrolysis J Mol Biol, 2006
Formation of a ternary complex between GM2 activator protein, GM2 ganglioside and hexosaminidase A Biochim Biophys Acta, 1997