Ceramide synthase 3

Definition:

Ceramide synthase that catalyzes the transfer of the acyl chain from acyl-CoA to a sphingoid base, with high selectivity toward very- and ultra-long-chain fatty acyl-CoA (chain length greater than C22). N-acylates sphinganine and sphingosine bases to form dihydroceramides and ceramides in de novo synthesis and salvage pathways, respectively. It is crucial for the synthesis of ultra-long-chain ceramides in the epidermis, to maintain epidermal lipid homeostasis and terminal differentiation.

References:

[1]. Richard Jennemann, et al. Loss of ceramide synthase 3 causes lethal skin barrier disruption. Hum Mol Genet. 2012 Feb 1;21(3):586-608. [Content Brief]

[2]. Franz P W Radner, et al. Mutations in CERS3 cause autosomal recessive congenital ichthyosis in humans. PLoS Genet. 2013 Jun;9(6):e1003536. [Content Brief]

[3]. Sujoy Lahiri, et al. Kinetic characterization of mammalian ceramide synthases: determination of K(m) values towards sphinganine. FEBS Lett. 2007 Nov 13;581(27):5289-94. [Content Brief]

[4]. Takayuki Sassa, et al. Enzyme Activities of the Ceramide Synthases CERS2-6 Are Regulated by Phosphorylation in the C-terminal Region. J Biol Chem. 2016 Apr 1;291(14):7477-87. [Content Brief]

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