Anabolic pathway of renal sulfatides

Anabolic pathway of renal sulfatides. SL synthesis starts out at the endoplasmic reticulum with the condensation of serine and a long chain acyl-CoA, in most cases palmitoyl-CoA. This is also the rate-limiting step, regulated by ORMDL proteins (ORM1-like proteins). After reduction of 3-keto-sphinganine to sphinganine, condensation with a variety of acyl-CoAs yields dihydroceramides, which are modified by dihydroceramid-Δ4-desaturase (Des)1 or Des2 to result in Cers or phyto-Cers, respectively, by that creating the huge diversity of Cer anchors found in renal sulfatides SM4s and SM3. Gray areas mark the structural changes in the molecules caused by the previous enzymatic step. 3-KSa-red., 3-ketosphinganine reductase; Sms 1 and 2, sphingomyelin synthase 1 and 2; LacCer-synthase, lactosylceramide synthase; Ugcg, UDP-glucose ceramide glucosyltransferase.

Publication

Renal sulfatides: sphingoid base-dependent localization and region-specific compensation of CerS2-dysfunction. (2014) Christian Marsching, et al. J Lipid Res. 2014 Nov;55(11):2354-2369. Figure: F1. Homo sapiens

Disease mentions