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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. .

Territories

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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,bHEXB hydrolyze PSAP(195-273):Gb4Cer:PE

H2O + PSAP(195-273):Gb4Cer:PE → GalNAc + PSAP(195-273):Gb3Cer:PE

Reactome: R-HSA-1605632
Compartment: lysosomal lumen
Rhea: None recorded

Inputs

H2OCHEBI:15377, H2O, lysosomal lumen
PSAP(195-273):Gb4Cer:PER-HSA-9840842, lysosomal lumen

Outputs

GalNAcCHEBI:28037, C8H15NO6, lysosomal lumen
PSAP(195-273):Gb3Cer:PER-HSA-9840848, lysosomal lumen

Catalysed by

beta-N-acetylhexosaminidase activity of bHEXA,bHEXB [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
Proteolytic processing of pro-alpha and pro-beta precursors from human beta-hexosaminidase. Generation of the mature alpha and beta a beta b subunits J Biol Chem, 1988
Crystal structure of human beta-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease J Mol Biol, 2003
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