In cells, fatty aldehydes are produced during oxidative stress-induced lipid peroxidation and by enzymatic degradation of a series of lipids including sphingosine-1-phosphate, plasmalogens, and alkylglycerols such as the platelet-activating factor. Impaired FALDH function has been shown to alter the metabolic profiles of connected pathways and can lead to the development of the SLS (dashed red arrows).
Reproduced with permission from: Keller MA, Zander U, Fuchs JE, et al. A gatekeeper helix determines the substrate specificity of Sjögren-Larsson Syndrome enzyme fatty aldehyde dehydrogenase. Nat Commun 2014; 5:4439. Copyright © 2014 Springer Nature.
https://www.nature.com/ncomms/.
