Target intelligence / Profile preview

Delta 4-desaturase, sphingolipid 1 (DEGS1)

Target
DEGS1
Molecular classification
Enzyme, Membrane fatty acid desaturase family, Integral membrane protein
01

Overview

Delta 4-desaturase, sphingolipid 1 (DEGS1) is an integral membrane enzyme belonging to the membrane fatty acid desaturase family. It is responsible for introducing a double bond at the Delta4 position during sphingolipid biosynthesis, specifically converting D‑erythro‑sphinganine to D‑erythro‑sphingosine. This reaction is essential for generating bioactive sphingoid bases that function as important signaling molecules in eukaryotic cells. The enzyme contains three histidine-rich motifs characteristic of this protein family and localizes primarily to the endoplasmic reticulum. Mutations or dysregulation in DEGS1 have been linked to rare neurodegenerative disorders such as hypomyelinating leukodystrophies. Its activity can be inhibited by certain natural products including curcumin and Δ9-tetrahydrocannabinol.

Other names
Sphingolipid delta(4)-desaturase DES1Degenerative spermatocyte homolog 1Degs1Des1
02

Mechanism of action

Inhibition of DEGS1 enzymatic activity by small molecules such as curcumin and THC reduces the formation of desaturated sphingolipids, potentially altering cell signaling and lipid homeostasis.

03

Biological functions

Sphingolipid metabolism (specifically, catalyzes the conversion of D-erythro-sphinganine to D-erythro-sphingosine)Insertion of double bonds into specific positions in fatty acidsRegulation of biosynthetic processing of other proteins (e.g., EGF receptor)Signal transduction via sphingolipids as signaling molecules
04

Disease associations

Leukodystrophy, hypomyelinating, 18Hypomyelinating leukodystrophy
05

Safety considerations

No notable safety concerns or therapeutic challenges are specifically reported in the provided sources.
06

Interacting drugs

Curcumin (natural product inhibitor)

1 more in the full profile.

07

Biomarkers

No established clinical biomarkers for patient selection or efficacy monitoring are reported in the provided sources.

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