EC - Sphingolipid C4-monooxygenase

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IntEnz Enzyme Nomenclature


Accepted name:
sphingolipid C4-monooxygenase
Other names:
sphingolipid C4-hydroxylase
SUR2 (gene name)
SBH1 (gene name)
SBH2 (gene name)
sphinganine C4-monooxygenase
DEGS2 (gene name)
Systematic name:
dihydroceramide,ferrocytochrome b5:oxygen oxidoreductase (C4-hydroxylating)



The enzyme, which belongs to the familiy of endoplasmic reticular cytochrome b5-dependent enzymes, is involved in the biosynthesis of sphingolipids in eukaryotes. Some enzymes are bifunctional and also catalyse EC, sphingolipid 4-desaturase [4].

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , UniPathway
Structural data: CSA , EC2PDB


  1. Haak, D., Gable, K., Beeler, T., Dunn, T.
    Hydroxylation of Saccharomyces cerevisiae ceramides requires Sur2p and Scs7p.
    J. Biol. Chem. 272 : 29704-29710 (1997). [PMID: 9368039]
  2. Grilley, M. M., Stock, S. D., Dickson, R. C., Lester, R. L., Takemoto, J. Y.
    Syringomycin action gene SYR2 is essential for sphingolipid 4-hydroxylation in Saccharomyces cerevisiae.
    J. Biol. Chem. 273 : 11062-11068 (1998). [PMID: 9556590]
  3. Sperling, P., Ternes, P., Moll, H., Franke, S., Zahringer, U., Heinz, E.
    Functional characterization of sphingolipid C4-hydroxylase genes from Arabidopsis thaliana.
    FEBS Lett. 494 : 90-94 (2001). [PMID: 11297741]
  4. Ternes, P., Franke, S., Zahringer, U., Sperling, P., Heinz, E.
    Identification and characterization of a sphingolipid delta 4-desaturase family.
    J. Biol. Chem. 277 : 25512-25518 (2002). [PMID: 11937514]
  5. Mizutani, Y., Kihara, A., Igarashi, Y.
    Identification of the human sphingolipid C4-hydroxylase, hDES2, and its up-regulation during keratinocyte differentiation.
    FEBS Lett. 563 : 93-97 (2004). [PMID: 15063729]

[EC created 2012 as EC, transferred 2015 to EC]