FIG 11.

A model for reduced acylceramide production in SLS. Acylceramides are produced from ω-OH ceramide and TG via a transacylation reaction (left). The LCB sphingosine is converted to trans-2-hexadecenal in the degradation pathway and further metabolized to ester-linked glycerolipids under normal conditions (right). Under FALDH-deficient conditions, such as ALDH3A2 mutation in humans (SLS) and Aldh3a2 Aldh3b2 DKO in mice, the accumulated trans-2-hexadecenal attacks and inhibits ABHD5, resulting in reduced acylceramide production.