Roles and regulation of secretory and lysosomal acid sphingomyelinase.

  title={Roles and regulation of secretory and lysosomal acid sphingomyelinase.},
  author={Russell William Jenkins and Daniel Canals and Yusuf A. Hannun},
  journal={Cellular signalling},
  volume={21 6},
Acid sphingomyelinase occupies a prominent position in sphingolipid catabolism, catalyzing the hydrolysis of sphingomyelin to ceramide and phosphorylcholine. Enzymatic dysfunction of acid sphingomyelinase results in Niemann-Pick disease, a lysosomal storage disorder characterized at the cellular level by accumulation of sphingomyelin within the endo-lysosomal compartment. Over the past decade interest in the role of acid sphingomyelinase has moved beyond its "housekeeping" function in… CONTINUE READING
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