Loading the map…

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. .

Territories

Select a territory to bring it forward.

Reading the map

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,bHEXB hydrolyze GM2A:GA2 to GM2A:LacCer

H2O + GM2A:GA2 → GalNAc + GM2A:LacCer

Reactome: R-HSA-9840833
Compartment: lysosomal lumen
Rhea: None recorded

Inputs

H2OCHEBI:15377, H2O, lysosomal lumen
GM2A:GA2R-HSA-9840792, lysosomal lumen

Outputs

GalNAcCHEBI:28037, C8H15NO6, lysosomal lumen
GM2A:LacCerR-HSA-9840837, lysosomal lumen

Catalysed by

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

Literature

Enzyme alterations and lipid storage in three variants of Tay-Sachs disease J Neurochem, 1971
Ganglioside Metabolism in Health and Disease Prog Mol Biol Transl Sci, 2018
Emerging concepts of ganglioside metabolism FEBS Lett, 2018