Skip to content

DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap

Data

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.

B4GALNT1 dimer transfers GalNAc to gangliosides

GD3,GM3,GT3,SM3,LacCer + UDP-GalNAc → GD2,GM2, GA2,GT2,SM2 + UDP + H+

Reactome: R-HSA-8856223
Compartment: Golgi membrane · Golgi lumen
Rhea: None recorded

Inputs

GD3,GM3,GT3,SM3,LacCerR-ALL-8856243, Golgi membrane
UDP-GalNAcCHEBI:57847, C17H25N3O17P2, Golgi lumen

Outputs

GD2,GM2, GA2,GT2,SM2R-ALL-8856242, Golgi membrane
UDPCHEBI:58223, C9H11N2O12P2, Golgi lumen
H+CHEBI:15378, H, Golgi lumen

Catalysed by

(N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase activity of B4GALNT1 dimer [Golgi membrane]EC 2.4.1.92

Literature

Disulfide bonds of GM2 synthase homodimers. Antiparallel orientation of the catalytic domains J. Biol. Chem., 2000
Alteration of ganglioside biosynthesis responsible for complex hereditary spastic paraplegia Am. J. Hum. Genet., 2013
Expression cloning of beta 1,4 N-acetylgalactosaminyltransferase cDNAs that determine the expression of GM2 and GD2 gangliosides J. Biol. Chem., 1992
Substrate specificity of beta 1,4-N-acetylgalactosaminyltransferase in vitro and in cDNA-transfected cells. GM2/GD2 synthase efficiently generates asialo-GM2 in certain cells J Biol Chem, 1995
Emerging concepts of ganglioside metabolism FEBS Lett, 2018