Acid sphingomyelinase

Acid sphingomyelinase is one of the enzymes that make up the sphingomyelinase (SMase) family, responsible for catalyzing the breakdown of sphingomyelin to ceramide and phosphorylcholine.[1] They are organized into alkaline, neutral, and acidic SMase depending on the pH in which their enzymatic activity is optimal. Acid sphingomyelinases' (aSMases) enzymatic activity can be influenced by drugs, lipids, cations, pH, redox and other proteins in the environment.[2][3][4] Specifically aSMases have been shown to have increased enzymatic activity in lysobisphosphatidic acid (LBPA) or phosphatidylinositol (PI) enriched environments, and inhibited activity when phosphorylated derivatives of PI are present.[4]

Sphingomyelin phosphodiesterase 1 (SMPD1) is the gene that codes for two aSMase enzymes distinct in the pools of sphingomyelin they hydrolyse.[4] Lysosomal sphingomyelinase (L-SMase) is found in the lysosomal compartment, and the secretory sphingomyelinase (S-SMase) is found extracellularly.

  1. ^ Schuchman, Edward H (May 2010). "Acid Sphingomyelinase, cell membranes and human disease: Lessons from Niemann-Pick disease". FEBS Letters. 584 (9): 1895–1900. Bibcode:2010FEBSL.584.1895S. doi:10.1016/j.febslet.2009.11.083. PMID 19944693.
  2. ^ Kornhuber J, Muehlbacher M, Trapp S, Pechmann S, Friedl A, Reichel M, Muehle C, Terfloth L, Groemer TW, Spitzer GM, Liedl KR, Gulbins E, Tribal P (2006). "Identification of novel functional inhibitors of acid sphingomyelinase". PLOS ONE. 6 (8): e23852. doi:10.1371/journal.pone.0023852. PMC 3166082. PMID 21909365.
  3. ^ McGovern, Mm; Schuchman EH (7 Dec 2006). "Acid Sphingomyelinase Deficiency". GeneReviews.
  4. ^ a b c Jenkins, RW; Canals D; Hannun YA (21 June 2009). "Roles and Regulation of Secretory and Lysosomal Acid Sphingomyelinases". Cell Signal. 21 (6): 836–846. doi:10.1016/j.cellsig.2009.01.026. PMC 3488588. PMID 19385042.