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Common structures of representative sphingolipids

Sphingolipids contain a long-chain base, which can be saturated (sphinganine) or monounsaturated at C4 (sphingosine). N-acylation at the amino group on C2 creates ceramide, which may consist of a varying number of carbon atoms. The representative ceramide shown is the 16-carbon palmitoyl-ceramide. The addition of a phosphocholine at C1 creates the parent phosphosphingolipid sphingomyelin, and glycosylation at the same carbon generates glucosylceramide. (Source: Abed Rabbo M, Khodour Y, Kaguni LS, Stiban J. Sphingolipid lysosomal storage diseases: from bench to bedside. Lipids Health Dis 2021;20[1]:44. Creative Commons Attribution 4.0 International License,

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