DEGS2 oxygenates dihydroceramide

Stable Identifier
R-HSA-428260
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
dihydroceramide + Fe2+ + H+ + O2 => phytoceramide + Fe3+ + H2O
ReviewStatus
5/5
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DEGS2 (sphingolipid C4-hydroxylase 2 / “degenerative spermatocyte homolog 2”) enzyme associated with the cytosolic face of the endoplasmic reticulum catalyzes the hydroxylation of dihydroceramide to form phytoceramide (Mizutani et al. 2004). DEGS2 appears not to be essential, with another unknown enzyme catalyzing the same reaction. The highest activity of DEGS2 is on long-chain (>= C21) dihydroceramides. DEGS2 also catalyzes the C4-dehydrogenation of dihydroceramide, but this activity is minor (Ota et al., 2023).

Literature References
PubMed ID Title Journal Year
15063729 Identification of the human sphingolipid C4-hydroxylase, hDES2, and its up-regulation during keratinocyte differentiation

Igarashi, Y, Kihara, A, Mizutani, Y

FEBS Lett 2004
36907437 Bifunctional DEGS2 has higher hydroxylase activity toward substrates with very-long-chain fatty acids in the production of phytosphingosine ceramides

Nojiri, K, Matsuda, J, Ota, A, Jojima, K, Kihara, A, Miyamoto, M, Naganuma, T, Morita, H

J Biol Chem 2023
Participants
Participates
Catalyst Activity

sphingosine hydroxylase activity of DEGS2 [endoplasmic reticulum membrane]

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