EC - Very-long-chain ceramide synthase

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IntEnz Enzyme Nomenclature


Accepted name:
very-long-chain ceramide synthase
Other names:
sphingoid base N-very-long-chain fatty acyl-coA transferase
mammalian ceramide synthase 2
Systematic name:
very-long-chain fatty acyl-coA:sphingoid base N-acyltransferase



This entry describes ceramide synthase enzymes that are specific for very-long-chain fatty acyl-CoA substrates. The two isoforms from yeast and the plant LOH1 and LOH3 isoforms transfer 24:0 and 26:0 acyl chains preferentially and use phytosphingosine as the preferred sphingoid base. The mammalian CERS2 isoform is specific for acyl donors of 20-26 carbons, which can be saturated or unsaturated. The mammalian CERS3 isoform catalyses this activity, but has a broader substrate range and also catalyses the activity of EC, ultra-long-chain ceramide synthase. Both mammalian enzymes can use multiple sphingoid bases, including sphinganine, sphingosine, and phytosphingosine.

Links to other databases

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


  1. Guillas, I., Kirchman, P. A., Chuard, R., Pfefferli, M., Jiang, J. C., Jazwinski, S. M., Conzelmann, A.
    C26-CoA-dependent ceramide synthesis of Saccharomyces cerevisiae is operated by Lag1p and Lac1p.
    EMBO J. 20 : 2655-2665 (2001). [PMID: 11387200]
  2. Pan, H., Qin, W. X., Huo, K. K., Wan, D. F., Yu, Y., Xu, Z. G., Hu, Q. D., Gu, K. T., Zhou, X. M., Jiang, H. Q., Zhang, P. P., Huang, Y., Li, Y. Y., Gu, J. R.
    Cloning, mapping, and characterization of a human homologue of the yeast longevity assurance gene LAG1.
    Genomics 77 : 58-64 (2001). [PMID: 11543633]
  3. Schorling, S., Vallee, B., Barz, W. P., Riezman, H., Oesterhelt, D.
    Lag1p and Lac1p are essential for the Acyl-CoA-dependent ceramide synthase reaction in Saccharomyces cerevisae.
    Mol. Biol. Cell 12 : 3417-3427 (2001). [PMID: 11694577]
  4. Mizutani, Y., Kihara, A., Igarashi, Y.
    Mammalian Lass6 and its related family members regulate synthesis of specific ceramides.
    Biochem. J. 390 : 263-271 (2005). [PMID: 15823095]
  5. Laviad, E. L., Albee, L., Pankova-Kholmyansky, I., Epstein, S., Park, H., Merrill, A. H., Futerman, A. H.
    Characterization of ceramide synthase 2: tissue distribution, substrate specificity, and inhibition by sphingosine 1-phosphate.
    J. Biol. Chem. 283 : 5677-5684 (2008). [PMID: 18165233]
  6. Imgrund, S., Hartmann, D., Farwanah, H., Eckhardt, M., Sandhoff, R., Degen, J., Gieselmann, V., Sandhoff, K., Willecke, K.
    Adult ceramide synthase 2 (CERS2)-deficient mice exhibit myelin sheath defects, cerebellar degeneration, and hepatocarcinomas.
    J. Biol. Chem. 284 : 33549-33560 (2009). [PMID: 19801672]

[EC created 2019]