EC - Ultra-long-chain ceramide synthase

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IntEnz Enzyme Nomenclature


Accepted name:
ultra-long-chain ceramide synthase
Other names:
mammalian ceramide synthase 3
sphingoid base N-ultra-long-chain fatty acyl-coA transferase
Systematic name:
ultra-long-chain fatty acyl-coA:sphingoid base N-acyltransferase



Mammals have six ceramide synthases that exhibit relatively strict specificity regarding the chain-length of their acyl-CoA substrates. Ceramide synthase 3 (CERS3) is the only enzyme that is active with ultra-long-chain acyl-CoA donors (C28 or longer). It is active in the epidermis, where its products are incorporated into acylceramides. CERS3 also accepts (2R)-2-hydroxy fatty acids and ω-hydroxy fatty acids, and can accept very-long-chain acyl-CoA substrates (see EC, very-long-chain ceramide synthase). It can use multiple sphingoid bases including sphinganine, sphingosine, phytosphingosine, and (6R)-6-hydroxysphingosine.

Links to other databases

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


  1. Mizutani, Y., Kihara, A., Igarashi, Y.
    LASS3 (longevity assurance homologue 3) is a mainly testis-specific (dihydro)ceramide synthase with relatively broad substrate specificity.
    Biochem. J. 398 : 531-538 (2006). [PMID: 16753040]
  2. Mizutani, Y., Kihara, A., Chiba, H., Tojo, H., Igarashi, Y.
    2-Hydroxy-ceramide synthesis by ceramide synthase family: enzymatic basis for the preference of FA chain length.
    J. Lipid Res. 49 : 2356-2364 (2008). [PMID: 18541923]
  3. Jennemann, R., Rabionet, M., Gorgas, K., Epstein, S., Dalpke, A., Rothermel, U., Bayerle, A., van der Hoeven, F., Imgrund, S., Kirsch, J., Nickel, W., Willecke, K., Riezman, H., Grone, H. J., Sandhoff, R.
    Loss of ceramide synthase 3 causes lethal skin barrier disruption.
    Hum. Mol. Genet. 21 : 586-608 (2012). [PMID: 22038835]
  4. Mizutani, Y., Sun, H., Ohno, Y., Sassa, T., Wakashima, T., Obara, M., Yuyama, K., Kihara, A., Igarashi, Y.
    Cooperative Synthesis of Ultra Long-Chain Fatty Acid and Ceramide during Keratinocyte Differentiation.
    PLoS ONE 8 : e67317 (2013). [PMID: 23826266]

[EC created 2019]