Compound

a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))

Glycosphingolipids which contain as their polar head group a lactose moiety bound in glycosidic linkage to the hydroxyl group of ceramide. Their accumulation in tissue, due to a defect in lactosylceramide beta-galactosidase, is the cause of lactosylceramidosis.

Definition from MeSH (D007790), National Library of Medicine, read 2026-09-23. NLM revises MeSH; this copy may not reflect its current record.

CHEBI:17950ChEBI

Open in the graph

Structure

2D depiction

Class: Organic compound
Formula: C31H56NO13R
Mass: 650.775 g/mol
Charge: 0
SMILES: *C(=O)N[C@@H](CO[C@@H]1O[C@H](CO)[C@@H](O[C@@H]2O[C@H](CO)[C@H](O)[C@H](O)[C@H]2O)[C@H](O)[C@H]1O)[C@H](O)/C=C/CCCCCCCCCCCCC
Look it up: ChEBI

Identity from ChEBI. Not evidence.

What it touches on the way

Mechanistic reach

9 entities

9 entities reached.

0 reported hops9 assembled from the scaffold

What the reference databases state

Statements

20 statements

20 statements · 2 reference databases · Subject: a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) · Sources: Human-GEM, Rhea

Order

a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is converted to beta-D-3-sulfogalactosyl-(1->4)-beta-D-glucosyl-(1<->1')-N-acylsphing-4-enineCompoundRhea RHEA:41736Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of A3GALT2ProteinRhea RHEA:42000Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of A4GALTProteinRhea RHEA:11924Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of A4GALTProteinHuman-GEM MAR08148Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of A4GALTProteinHuman-GEM MAR00801Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of B3GNT5ProteinHuman-GEM MAR08275Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of B3GNT5ProteinRhea RHEA:13905Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of B3GNT5ProteinHuman-GEM MAR00859Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of B4GALNT1ProteinRhea RHEA:47564Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of B4GALNT1ProteinHuman-GEM MAR00815Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of B4GALNT1ProteinHuman-GEM MAR08165Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of GAL3ST1ProteinHuman-GEM MAR00928Inferred only

Show the remaining 8

a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of GAL3ST1ProteinRhea RHEA:41736Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of GLB1ProteinHuman-GEM MAR00786Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of GLB1LProteinHuman-GEM MAR00786Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of LCTProteinHuman-GEM MAR00786Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of ST3GAL5ProteinRhea RHEA:18417Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of ST3GAL5ProteinHuman-GEM MAR08184Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of ST3GAL5ProteinHuman-GEM MAR00824Inferred only
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) is a substrate of TMPPEProteinHuman-GEM MAR00786Inferred only

Reactions

37 reactions

D-glucosyl-N-acylsphingosine+CHEBI:67119proton+a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))+UDP
Catalysed by GLT8D1 or B4GALT7 or PIGV or B4GALT1 or GALNT7 or B4GALT2 or A4GNT or B4GALT6 or PIGZ or GLT8D2 or B4GALT4 or GCNT7 and 23 more alternatives
Reaction pageLeft to rightBalance undeterminableEC 2.4.1.-endoplasmic reticulum
Human-GEM MAR00765CC BY 4.0Reference data
alpha-D-galactose+D-glucosyl-N-acylsphingosinewater+a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))
Catalysed by LCT or GLB1L or GLB1
Reaction pageLeft to rightBalance undeterminableEC 3.2.1.23extracellular space
Human-GEM MAR00766CC BY 4.0Reference data
water+a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))alpha-D-galactose+D-glucosyl-N-acylsphingosine
Catalysed by LCT & GLB1 & GLB1L & TMPPE
Reaction pageLeft to rightBalance undeterminableEC 3.2.1.23lysosome
Human-GEM MAR00786CC BY 4.0Reference data
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))+CHEBI:67119a globoside Gb3Cer (d18:1(4E))+proton+UDP
Catalysed by A4GALT
Reaction pageLeft to rightBalance undeterminableEC 2.4.1.228cytosol
Human-GEM MAR00801CC BY 4.0Reference data
a globoside Gb3Cer (d18:1(4E))+wateralpha-D-galactose+a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))
Catalysed by GLA
Reaction pageLeft to rightBalance undeterminableEC 3.2.1.22cytosol
Human-GEM MAR00803CC BY 4.0Reference data
a globoside Gb3Cer (d18:1(4E))+wateralpha-D-galactose+a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))
Catalysed by HEXB or HEXD or HEXA
Reaction pageLeft to rightBalance undeterminableEC 3.2.1.52lysosome
Human-GEM MAR00804CC BY 4.0Reference data
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))+MAM03110ca ganglioside GA2 (d18:1(4E))+proton+UDP
Catalysed by B4GALNT1
Reaction pageLeft to rightBalance undeterminableEC 2.4.1.92cytosol
Human-GEM MAR00815CC BY 4.0Reference data
a ganglioside GA2 (d18:1(4E))+watera beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))+MAM02525c
Catalysed by HEXB or HEXD or HEXA
Reaction pageLeft to rightBalance undeterminableEC 3.2.1.52cytosol
Human-GEM MAR00821CC BY 4.0Reference data
CMP-N-acetyl-beta-neuraminate+a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))cytidine 5'-monophosphate+a ganglioside GM3 (d18:1(4E))+proton
Catalysed by ST3GAL5
Reaction pageLeft to rightBalance undeterminableEC 2.4.3.9cytosol
Human-GEM MAR00824CC BY 4.0Reference data
a ganglioside GM3 (d18:1(4E))+watera beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))+N-acetyl-beta-neuraminic acid
Catalysed by NEU2 or NEU3 or NEU4 or NEU1
Reaction pageLeft to rightBalance undeterminableEC 3.2.1.18cytosol
Human-GEM MAR00825CC BY 4.0Reference data
a ganglioside GM3 (d18:1(4E))+watera beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))+N-acetyl-beta-neuraminic acid
Catalysed by NEU2 or NEU3 or NEU4 or NEU1
Reaction pageLeft to rightBalance undeterminableEC 3.2.1.18lysosome
Human-GEM MAR00826CC BY 4.0Reference data
a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))+CHEBI:16264proton+a beta-D-GlcNAc-(1->3)-beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))+UDP
Catalysed by B3GNT5
Reaction pageLeft to rightBalance undeterminableEC 2.4.1.206cytosol
Human-GEM MAR00859CC BY 4.0Reference data

Show the remaining 25

Other names

30 names

Resolves to: a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))

Also called: (Gal)1 (Glc)1 (Cer)1beta-D-Gal-(1->4)-beta-D-Glc-(11)-Cerbeta-D-galactosyl-(1->4)-beta-D-glucosyl-(11)-ceramidebeta-D-galactosyl-(1->4)-beta-D-glucosylceramidesbeta-D-Galactosyl-1,4-beta-D-glucosylceramidebeta-D-galactosyl-1,4-beta-D-glucosylceramidesbeta-D-Galp-(1->4)-beta-D-Glc-(1)-Cerbeta-lactosylceramideD-Galactosyl-1,4-beta-D-glucosylceramideGal-beta1->4Glc-beta1->1'CerGalbeta1-4Glcbeta-CerGalβ1-4Glcβ-CerLacCerLacCer(d18:1(4E))Lactosyl ceramide (d18:1(4E))Lactosyl-N-acylsphingosineLactosylceramidelactosylceramidesN-lignoceroyl-1-sphingosyl lactosideβ-D-Gal-(1→4)-β-D-Glc-(1↔1)-Cerβ-D-galactosyl-(1→4)-β-D-glucosyl-(1↔1)-ceramideβ-D-galactosyl-(1→4)-β-D-glucosylceramidesβ-D-galactosyl-1,4-β-D-glucosylceramideβ-D-galactosyl-1,4-β-D-glucosylceramidesβ-D-Galp-(1→4)-β-D-Glc-(1↔)-Cerβ-lactosylceramide
Systematic name: (2S,3R,4E)-2-alkanamido-3-hydroxyoctadec-4-en-1-yl 4-O-beta-D-galactopyranosyl-beta-D-glucopyranoside(2S,3R,4E)-2-alkanamido-3-hydroxyoctadec-4-en-1-yl 4-O-β-D-galactopyranosyl-β-D-glucopyranosideN-[(2S,3R,4E)-1-{[beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl]oxy}-3-hydroxyoctadec-4-en-2-yl]alkanamideN-[(2S,3R,4E)-1-{[β-D-galactopyranosyl-(1→4)-β-D-glucopyranosyl]oxy}-3-hydroxyoctadec-4-en-2-yl]alkanamide