Phytosphingosine degradation pathway includes fatty acid Î±-oxidation reactions in the endoplasmic reticulum.

Proc. Natl. Acad. Sci. U.S.A.2017 Mar 28;114(13):E2616-E2623. Epub 2017 Mar 13

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-deficient cells, the unmetabolized fatty aldehydes are reduced to fatty alcohols and are incorporated into ether-linked glycerolipids. We also identify HACL2 (2-hydroxyacyl-CoA lyase 2) [previous name, ILVBL; ilvB (bacterial acetolactate synthase)-like] as the major 2-OH acyl-CoA lyase involved in the cleavage (C1 removal) reaction in the FA Î±-oxidation of the PHS degradation pathway. HACL2 is localized in the endoplasmic reticulum. Thus, in addition to the already-known FA Î±-oxidation in the peroxisomes, we have revealed the existence of FA Î±-oxidation in the endoplasmic reticulum in mammals.

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Phytosphingosine degradation pathway includes fatty acid Î±-oxidation reactions in the endoplasmic reticulum.

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