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Schematic representation of the de novo synthesis of sphingolipids

The acylchain is depicted in blue and can range from C14 to C26, depending on the CERS enzyme involved in acyl chain condensation (six CERS members exist). Depending on the acyl-CoA moiety used by SPT (ie, myristoyl-CoA, palmitoyl-CoA, or stearyl-CoA), different sphingoid bases can be produced (C16:1, C18:1, or C20:1).

Abbreviations: CERS, ceramide synthase; DES, desaturase; KDR, ketoreductase; SPT, serine palmitoyltransferase.

(Source: van Eijk M, Ferraz MJ, Boot RG, Aerts JM. Lyso-glycosphingolipids: presence and consequences. Essays Biochem 2020;64[3]:565-78. Creative Commons Attribution 4.0 International [CC BY 4.0] license, creativecommons.org/licenses/by/4.0.)

Associated Disorders

  • Adult globoid cell leukodystrophy
  • Diffuse brain-sclerosis
  • Diffuse gliosis
  • Early infantile globoid cell leukodystrophy
  • Juvenile globoid cell leukodystrophy
  • Late infantile globoid cell leukodystrophy
  • Leukodystrophy