Glycosphingolipid metabolism (Homo sapiens)

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4632752SM4GM3B3GALT2B4GALNT1Gala-SeriesB4GALT4GBA3A3GALT2CeramideGalCerGBA1Globo seriesB3GNT5GA2A4GALTB4GALT6B4GALT5nLc4CerUGT8LacCeriGb3Lc3CerGLAB4GALT3Gb3B4GALT1GlcCerUGCGCDGGanglio seriesGBA2SM3Neolacto seriesNEU4GM4B3GALT1LM1NEU3Lacto seriesGAL3ST1A4GALTGALCARSA1ST3GAL57GAL3ST1ST3GAL5isoGlobo series


Glycosphingolipid metabolism, based on sphingomap. Initial top level pathway

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Ontology Terms



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  1. Yamaguchi K, Hata K, Koseki K, Shiozaki K, Akita H, Wada T, Moriya S, Miyagi T; ''Evidence for mitochondrial localization of a novel human sialidase (NEU4).''; Biochem J, 2005 PubMed Europe PMC Scholia
  2. Honke K, Yamane M, Ishii A, Kobayashi T, Makita A; ''Purification and characterization of 3'-phosphoadenosine-5'-phosphosulfate:GalCer sulfotransferase from human renal cancer cells.''; J Biochem, 1996 PubMed Europe PMC Scholia
  3. Wenger DA, Sattler M, Hiatt W; ''Globoid cell leukodystrophy: deficiency of lactosyl ceramide beta-galactosidase.''; Proc Natl Acad Sci U S A, 1974 PubMed Europe PMC Scholia
  4. Merril AH Jr; ''SphinGOMAP--a web-based biosynthetic pathway map of sphingolipids and glycosphingolipids.''; Glycobiology, 2005 PubMed Europe PMC Scholia
  5. Keusch JJ, Manzella SM, Nyame KA, Cummings RD, Baenziger JU; ''Expression cloning of a new member of the ABO blood group glycosyltransferases, iGb3 synthase, that directs the synthesis of isoglobo-glycosphingolipids.''; J Biol Chem, 2000 PubMed Europe PMC Scholia
  6. Yamashiro S, Haraguchi M, Furukawa K, Takamiya K, Yamamoto A, Nagata Y, Lloyd KO, Shiku H; ''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 PubMed Europe PMC Scholia
  7. Kase R, Bierfreund U, Klein A, Kolter T, Utsumi K, Itoha K, Sandhoff K, Sakuraba H; ''Characterization of two alpha-galactosidase mutants (Q279E and R301Q) found in an atypical variant of Fabry disease.''; Biochim Biophys Acta, 2000 PubMed Europe PMC Scholia


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125119view10:00, 24 January 2023Conroy lipidsadd ECs
124465view20:09, 29 October 2022EgonwCompleted the LM identifier
124460view07:49, 29 October 2022EgonwUpdated the UniProt data sources
124453view08:41, 28 October 2022Conroy lipids
124452view08:38, 28 October 2022Conroy lipids
124451view08:25, 28 October 2022Conroy lipids
124444view15:39, 27 October 2022Conroy lipids
124443view15:19, 27 October 2022Conroy lipids
124442view15:16, 27 October 2022Conroy lipidsfix broken links
124441view15:15, 27 October 2022Conroy lipidsfix non-touching nodes
124440view15:12, 27 October 2022Conroy lipidsupdate layout, add some back-reactions
124398view15:38, 25 October 2022Conroy lipidsfix unconnections
124397view15:34, 25 October 2022Conroy lipidsOntology Term : 'sphingolipid biosynthetic pathway' added !
124396view15:34, 25 October 2022Conroy lipidsOntology Term : 'ganglioside metabolic pathway' added !
124395view15:30, 25 October 2022Conroy lipidsadded some more data. Need to clean up layout considerably
124393view11:19, 25 October 2022Conroy lipidsNew pathway

External references


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NameTypeDatabase referenceComment
A3GALT2GeneProductU3KPV4 (Uniprot-TrEMBL)
A4GALTGeneProductQ9NPC4 (Uniprot-TrEMBL)
ARSAGeneProductP15289 (Uniprot-TrEMBL)
B3GALT1GeneProductQ9Y5Z6 (Uniprot-TrEMBL)
B3GALT2GeneProductO43825 (Uniprot-TrEMBL)
B3GNT5GeneProductQ9BYG0 (Uniprot-TrEMBL)
B4GALNT1GeneProductQ00973 (Uniprot-TrEMBL)
B4GALT1GeneProductO60513 (Uniprot-TrEMBL)
B4GALT3GeneProductO60512 (Uniprot-TrEMBL)
B4GALT4GeneProductO60513 (Uniprot-TrEMBL)
B4GALT5GeneProductO43286 (Uniprot-TrEMBL)
B4GALT6GeneProductQ9UBX8 (Uniprot-TrEMBL)
CDGMetaboliteLMSP0509AA00 (LIPID MAPS)
CeramideMetaboliteLMSP02010000 (LIPID MAPS)
GA2MetaboliteLMSP0503AA00 (LIPID MAPS)
GAL3ST1GeneProductQ99999 (Uniprot-TrEMBL)
GALCGeneProductP54803 (Uniprot-TrEMBL)
GBA1GeneProductP04062 (Uniprot-TrEMBL)
GBA2GeneProductQ9HCG7 (Uniprot-TrEMBL)
GBA3GeneProductQ9H227 (Uniprot-TrEMBL)
GLAGeneProductP06280 (Uniprot-TrEMBL)
GM3MetaboliteLMSP0601AJ00 (LIPID MAPS)
GM4MetaboliteLMSP0601AA00 (LIPID MAPS)
GalCerMetaboliteLMSP0501AC00 (LIPID MAPS)
Ganglio seriesPathway
Gb3MetaboliteLMSP0502AA00 (LIPID MAPS)
GlcCerMetaboliteLMSP0501AA00 (LIPID MAPS)
Globo seriesPathway
LM1MetaboliteLMSP0504AB00 (LIPID MAPS)
LacCerMetaboliteLMSP0501AB00 (LIPID MAPS)
Lacto seriesPathway
Lc3CerMetaboliteLMSP0504AA00 (LIPID MAPS)
NEU3GeneProductQ9UQ49 (Uniprot-TrEMBL)
NEU4GeneProductQ8WWR8 (Uniprot-TrEMBL)
Neolacto seriesPathway
SM3MetaboliteLMSP0602AA00 (LIPID MAPS)
SM4MetaboliteLMSP0602AN00 (LIPID MAPS)
ST3GAL5GeneProductQ9UNP4 (Uniprot-TrEMBL)
UGCGGeneProductQ16739 (Uniprot-TrEMBL)
UGT8GeneProductQ16880 (Uniprot-TrEMBL)
iGb3MetaboliteLMSP0506AQ00 (LIPID MAPS)
isoGlobo seriesPathway
nLc4CerMetaboliteLMSP0505AA00 (LIPID MAPS)

Annotated Interactions

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SourceTargetTypeDatabase referenceComment
CeramideGalCermim-conversion13094 (Rhea) EC:
CeramideGlcCermim-conversion12089 (Rhea) EC:
GM3LacCermim-conversion47901 (Rhea) EC:
GalCerCDGmim-conversion60061 (Rhea)
GalCerCeramidemim-conversion14298 (Rhea) EC:
GalCerGM4mim-conversion47601 (Rhea) EC:
GalCerSM4mim-conversion20614 (Rhea) EC:
Gb3LacCermim-conversion21113 (Rhea) EC:
GlcCerCeramidemim-conversion13270 (Rhea) EC:
GlcCerLacCermim-conversion31496 (Rhea) EC:
LacCerGA2mim-conversion47565 (Rhea) EC:
LacCerGM3mim-conversion18418 (Rhea) EC:
LacCerGlcCermim-conversion48061 (Rhea)
LacCerLc3Cermim-conversion13906 (Rhea) EC:
LacCerSM3mim-conversion41737 (Rhea) EC:
LacCeriGb3mim-conversion42001 (Rhea) EC:
LacCermim-conversion11925 (Rhea) EC:
Lc3CerLM1mim-conversion16046 (Rhea) EC:
Lc3CernLc4Cermim-conversion31500 (Rhea) EC:
SM4GalCermim-conversion21301 (Rhea) EC:
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