Fatty acid transport protein 4 is required for incorporation of saturated ultralong-chain fatty acids into epidermal ceramides and monoacylglycerols

Sci Rep. 2019 Sep 13;9(1):13254. doi: 10.1038/s41598-019-49684-y.

Abstract

Fatty acid transport protein 4 (FATP4) is an acyl-CoA synthetase that is required for normal permeability barrier in mammalian skin. FATP4 (SLC27A4) mutations cause ichthyosis prematurity syndrome, a nonlethal disorder. In contrast, Fatp4-/- mice die neonatally from a defective barrier. Here we used electron microscopy and lipidomics to characterize defects in Fatp4-/- mice. Mutants showed lamellar body, corneocyte lipid envelope, and cornified envelope abnormalities. Lipidomics identified two lipids previously speculated to be present in mouse epidermis, sphingosine β-hydroxyceramide and monoacylglycerol; mutants displayed decreased proportions of these and the two ceramide classes that carry ultralong-chain, amide-linked fatty acids (FAs) thought to be critical for barrier function, unbound ω-O-acylceramide and bound ω-hydroxyceramide, the latter constituting the major component of the corneocyte lipid envelope. Other abnormalities included elevated amounts of sphingosine α-hydroxyceramide, phytosphingosine non-hydroxyceramide, and 1-O-acylceramide. Acyl chain length alterations in ceramides also suggested roles for FATP4 in esterifying saturated non-hydroxy and β-hydroxy FAs with at least 25 carbons and saturated or unsaturated ω-hydroxy FAs with at least 30 carbons to CoA. Our lipidomic analysis is the most thorough such study of the Fatp4-/- mouse skin barrier to date, providing information about how FATP4 can contribute to barrier function by regulating fatty acyl moieties in various barrier lipids.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Ceramides / metabolism*
  • Fatty Acid Transport Proteins / physiology*
  • Fatty Acids / metabolism*
  • Keratinocytes / metabolism*
  • Lipids / analysis
  • Mice
  • Mice, Transgenic
  • Monoglycerides / metabolism*
  • Skin / metabolism*

Substances

  • Ceramides
  • Fatty Acid Transport Proteins
  • Fatty Acids
  • Lipids
  • Monoglycerides
  • Slc27a1 protein, mouse
  • Slc27a4 protein, mouse