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Sphingolipid Synthesis Enzymes (N/A)
- Target
- N/A
- Molecular classification
- Enzyme
01
Overview
Sphingolipid synthesis enzymes are a group of membrane-associated proteins responsible for the de novo biosynthesis of sphingolipids. These enzymes are crucial for cell membrane integrity, signal transduction, and cell recognition processes. Key enzymes include Serine Palmitoyltransferase (SPT), 3-Ketodihydrosphingosine Reductase, Ceramide Synthases (CerS), and Dihydroceramide Desaturase. Disruption in their function can lead to severe developmental defects or diseases known as sphingolipidoses.
- Other names
- SPTSerine Palmitoyltransferase3-Ketodihydrosphingosine ReductaseCeramide SynthasesCerSDihydroceramide DesaturaseGlucosylceramide SynthaseGalactosylceramide SynthaseSphingomyelin Synthase
02
Mechanism of action
SKI II inhibits sphingosine kinases (SK1/2) and desaturases involved upstream/downstream in this pathway; Fingolimod (FTY720) acts on downstream metabolites but also inhibits certain Cer synthases at higher concentrations
03
Biological functions
Cell membrane integritySignal transductionCell recognitionLipid metabolism
04
Disease associations
SphingolipidosesGaucher diseaseTay-Sachs diseaseNiemann-Pick diseaseMetabolic disorders
05
Safety considerations
Embryonic lethality (loss-of-function mutations in some enzymes)Developmental defects
06
Interacting drugs
SKI II
1 more in the full profile.
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