Skip to content

DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap

Data

Loading the map…

Human metabolism

Reactome's human metabolism drawn as one network. Each molecule has one home node; widely shared molecules also appear as dashed copies in other territories, linked from their details. .

Territories

Select a territory to bring it forward.

Reading the map

Metabolite; its structure appears as you zoom in
Reaction, arrow toward the product
Cofactors and co-substrates are written beside the reaction when you zoom in
Dashed ring: a copy of a widely shared molecule or cofactor in another territory or pathway
Compartment transfer of the molecule it is linked to
Binding, release or complex event; its proteins are named in its details
Outline: reactions mostly in the mitochondrion

Arrows follow Reactome's curated reaction direction.

DEGS1 dehydrogenates dihydroceramide

2 H+ + O2 + DHCE + 2 CYB5B:Fe2+ → 2 H2O + (4E)-CERA + 2 CYB5B:Fe3+

Reactome: R-HSA-428259
Compartment: cytosol · endoplasmic reticulum membrane
Rhea: None recorded

Inputs

2 H+CHEBI:15378, H, cytosol
O2CHEBI:15379, O2, cytosol
DHCECHEBI:139048, C5H9NO3R2, endoplasmic reticulum membrane
2 CYB5B:Fe2+R-HSA-9842697, endoplasmic reticulum membrane

Outputs

2 H2OCHEBI:15377, H2O, cytosol
(4E)-CERACHEBI:195634, C6H9NO3R2, endoplasmic reticulum membrane
2 CYB5B:Fe3+R-HSA-9842694, endoplasmic reticulum membrane

Catalysed by

sphingolipid delta-4 desaturase activity of DEGS1 [endoplasmic reticulum membrane]EC 1.14.19.17; UniProt O15121

Literature

Characterization of ceramide synthesis. A dihydroceramide desaturase introduces the 4,5-trans-double bond of sphingosine at the level of dihydroceramide J Biol Chem, 1997
Identification and characterization of a sphingolipid delta 4-desaturase family J Biol Chem, 2002
The product of the MLD gene is a member of the membrane fatty acid desaturase family: overexpression of MLD inhibits EGF receptor biosynthesis Biochemistry, 1997
Dihydroceramide desaturase and dihydrosphingolipids: debutant players in the sphingolipid arena Prog Lipid Res, 2012
Conversion of dihydroceramide into ceramide: involvement of a desaturase Biochem J, 1997
DEGS1 -related leukodystrophy: a clinical report and review of literature Clin Dysmorphol, 2023