Path ID: DB00419_MESH_D052556_1
Concepts
| Identifier | Name | Type |
|---|---|---|
| MESH:C059896 | miglustat | Drug |
| UniProt:Q16739 | Ceramide glucosyltransferase | Protein |
| GO:0006679 | glucosylceramide biosynthetic process | BiologicalProcess |
| CHEBI:36500 | glucosylceramide | ChemicalSubstance |
| HP:0000707 | Abnormality of the nervous system | PhenotypicFeature |
| MESH:D052556 | Niemann-Pick disease, type C | Disease |
Relationships
NOTE: predicates are annotated in Biolink Model (v1.3.0)
| Subject | Predicate | Object |
|---|---|---|
| Miglustat | DECREASES ACTIVITY OF | Ceramide Glucosyltransferase |
| Ceramide Glucosyltransferase | POSITIVELY REGULATES | Glucosylceramide Biosynthetic Process |
| Glucosylceramide Biosynthetic Process | INCREASES ABUNDANCE OF | Glucosylceramide |
| Glucosylceramide | POSITIVELY CORRELATED WITH | Abnormality Of The Nervous System |
| Abnormality Of The Nervous System | MANIFESTATION OF | Niemann-Pick Disease, Type C |
Comment: Miglustat is an example of a substrate reduction therapy and it inhibits the synthesis of glucosylceramide (also known as glucocerebroside).
Reference:
- https://go.drugbank.com/drugs/DB00419
- https://pubmed.ncbi.nlm.nih.gov/30111334/
- https://en.wikipedia.org/wiki/Niemann%E2%80%93Pick_disease,_type_C#Other_treatments_under_investigation
- https://en.wikipedia.org/wiki/Miglustat#Mechanism_of_action
- https://en.wikipedia.org/wiki/Substrate_reduction_therapy