Compound

a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine

Cerebrosides which contain as their polar head group a galactose moiety bound in glycosidic linkage to the hydroxyl group of ceramide. Their accumulation in tissue, due to a defect in beta-galactosidase, is the cause of galactosylceramide lipidosis or globoid cell leukodystrophy.

Definition from MeSH (D005699), National Library of Medicine, read 2026-09-23. NLM revises MeSH; this copy may not reflect its current record.

CHEBI:18390ChEBI

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Structure

2D depiction

Class: Organic compound
Formula: C25H46NO8R
Mass: 488.636 g/mol
Charge: 0
SMILES: *C(=O)N[C@@H](CO[C@@H]1O[C@H](CO)[C@H](O)[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

10 entities

10 entities reached.

0 reported hops10 assembled from the scaffold

What the reference databases state

Statements

14 statements

14 statements · 1 reference database · Subject: a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine · Sources: Rhea

Order

a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is converted to an N-acyl-1-beta-D-(3-O-sulfo)-galactosyl-sphing-4-enineCompoundRhea RHEA:20613Inferred only
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is a substrate of GAL3ST1ProteinRhea RHEA:20613Inferred only
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is a substrate of GALCProteinRhea RHEA:14297Inferred only
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is a substrate of GBA1ProteinRhea RHEA:14297Inferred only
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is a substrate of GBA1ProteinRhea RHEA:70235Inferred only
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is a substrate of GBA2ProteinRhea RHEA:14297Inferred only
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is a substrate of GBA2ProteinRhea RHEA:70235Inferred only
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is a substrate of GBA3ProteinRhea RHEA:14297Inferred only
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is a substrate of ST3GAL5ProteinRhea RHEA:47600Inferred only
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is transported by ANO3ProteinRhea RHEA:38899Inferred only
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is transported by ANO4ProteinRhea RHEA:38899Inferred only
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is transported by ANO6ProteinRhea RHEA:38899Inferred only

Show the remaining 2

a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is transported by ANO7ProteinRhea RHEA:38899Inferred only
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine is transported by ANO9ProteinRhea RHEA:38899Inferred only

Reactions

8 reactions

an N-acylsphing-4-enine+UDP-alpha-D-galactosea beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine+UDP+hydron
Catalysed by UGT8
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:13093CC BY 4.0Reference data
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine+waterD-galactose+an N-acylsphing-4-enine
Catalysed by GBA1 or GALC or GBA3 or GBA2
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:14297CC BY 4.0Reference data
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine+3'-phosphoadenylyl sulfatean N-acyl-1-beta-D-(3-O-sulfo)-galactosyl-sphing-4-enine+adenosine 3',5'-bisphosphate+hydron
Catalysed by GAL3ST1
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:20613CC BY 4.0Reference data
an N-acyl-1-beta-D-(3-O-sulfo)-galactosyl-sphing-4-enine+watera beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine+sulfate+hydron
Catalysed by ARSA
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:21300CC BY 4.0Reference data
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-eninea beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine
Transported by ANO9 or ANO4 or ANO6 or ANO7 or ANO3
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:38899CC BY 4.0Reference data
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine+CMP-N-acetyl-beta-neuraminatea ganglioside GM4 (d18:1(4E))+CMP+hydron
Catalysed by ST3GAL5
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:47600CC BY 4.0Reference data
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine+UDP-alpha-D-galactoseCHEBI:143595+UDP+hydron
Reaction pageDirection not assertedBalance undeterminable
Rhea RHEA:60060CC BY 4.0Reference data
a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine+cholesterolcholesteryl 3-beta-D-galactoside+an N-acylsphing-4-enine
Catalysed by GBA1 or GBA2
Reaction pageReversibleBalance undeterminable
Rhea RHEA:70235CC BY 4.0Reference data

Where it reaches

10 systems, 16 regions, 43 tissues, 45 transporter routes — routes, not measurements: transporters known to carry a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine are expressed there, and no source reports it arriving.

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Nervous

Endocrine

Respiratory

Cardiovascular

Digestive

Urinary

Reproductive

Blood and immune

Musculoskeletal

Skin and adipose

Other names

20 names

Resolves to: a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine

Also called: 1-O-beta-D-galactopyranosylceramide1-O-D-galactopyranosyl-2-N-acylsphingosine1-O-β-D-galactopyranosylceramidebeta-galactosyl-(1->1)-ceramidebeta-GalCerCerebrosideD-Galactosyl-N-acylsphingosineD-GalactosylceramideGalactocerebrosidegalactocerebrosidesGalactosyl ceramideGalactosyl ceramide (d18:1(4E))GalactosylceramideGalbeta-CerGalCGalCerGalCer(d18:1(4E))ganglioside GL1bβ-galactosyl-(1→1)-ceramideβ-GalCer