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Generation of ceramide derived species including glycosphingolipids

Abbreviations: CDase, ceramidase; Cer1P, ceramide-1-phosphate; CGS, cerebroside sulfotransferase; CGT, UDP-galactose ceramide galactosyl transferase; CK, ceramide kinase; GCS, glucosylceramide synthase; GalCer, galactosylceramide; GlcCer, glucosylceramide; Hexa, hexadecanal; LacCer, lactosylceramide; LCS, lactosylceramide synthase; PE, phosphatidylethanolamine; S1P, sphingosine-1-phosphate; S1PL, S1P lyase; SK, sphingosine kinase; SMS, sphingomyelin synthase; SO, sphingosine.

(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,

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