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- W4366171164 abstract "Loss-of-function mutations in patatin-like phospholipase domain-containing protein 1 (PNPLA1) cause autosomal recessive congenital ichthyosis. Altered PNPLA1 activity is also implicated in the pathogenesis of atopic dermatitis and other common skin diseases. PNPLA1 has been proposed to synthesizeesterified omega-hydroxy acylsphingosine (EOS) and related acylceramides and acylacids, a unique family of epidermal barrier lipids that are enriched in the essential fatty acid linoleate. To test this hypothesis, we expressed and purified a soluble, truncated form of PNPLA1 in E. coli, along with the related protein PNPLA2 (ATGL, adipose triglyceride lipase), and co-activator CGI-58. Lipid substrates were prepared as liposomes, incubated with recombinant enzymes for 0.5 – 24 h and products were analyzed by HPLC-UV and LC-MS in comparison to authentic lipid standards. PNPLA1, like ATGL, catalyzed CGI-58-dependent lipolysis and acyltransferase reactions using triglyceride or diglyceride substrates. These reactions were rapid (1 – 2 h), with quantitative conversion (nmols per reaction) into free fatty acid and monoglyceride. Both PNPLA1 and ATGL also transferred acyl groups from glycerides to omega-hydroxy ceramide and omega-hydroxy fatty acid acceptors to form EOS and acyl acid, respectively, albeit in smaller quantities (pmols per reaction). PNPLA1 formed EOS more efficiently than ATGL, and exhibited a selectivity for the transfer of linoleate over oleate. Triglyceride or diglyceride was effective as acyl donor, and omega-hydroxy fatty acid, omega-hydroxy ceramide or glucosyl omega-hydroxy ceramide were effective as acceptor. These results support the hypothesis that PNPLA1 is involved in the synthesis of EOS and other acylceramides and acylacids in epidermis and suggest that the enrichment of these lipids with linoleic acid could result in part from the substrate selectivity of PNPLA1." @default.
- W4366171164 created "2023-04-19" @default.
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- W4366171164 date "2023-05-01" @default.
- W4366171164 modified "2023-09-28" @default.
- W4366171164 title "773 Purified recombinant PNPLA1 catalyzes the synthesis of acylceramides and acylacids of the mammalian skin barrier" @default.
- W4366171164 doi "https://doi.org/10.1016/j.jid.2023.03.782" @default.
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