Fig. 1.
Sphingolipid pathway and subcellular localization of sphingolipid enzymes. The biosynthesis of sphingolipids begins in the endoplasmatic reticulum (ER)–Golgi network. Vesicular transport distributes sphingolipids to different compartments such as plasma membrane, and lysosome. Sphingolipids and a sphingomyelinase (Man) have been found in the mitochondrion. a/nSMase acid/neutral sphingonyelinase, a/nCDase acid/neutral ceramidase, nSMase neutral sphingomyelinase, SK1/2 sphingosine kinase 1/2, Man mitochondrial sphingomyelinase, GBA acid β-glucosidase, SMS sphingomyelinase synthase, DES dihydroceramide desaturase, GCS glucosylceramide synthase, SPT serine palmitoyl-CoA transferase