SGMS2 transfers phosphocholine onto ceramide

Stable Identifier
Reaction [transition]
Homo sapiens
phosphatidylcholine + ceramide <=> sphingomyelin + diacylglycerol [SGMS2]
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SGMS2 (sphingomyelin synthase 2) catalyzes the reversible reaction of phosphatidylcholine and ceramide to form sphingomyelin and diacylglycerol. Most SGMS2 activity is associated with the plasma membrane, although active enzyme is also present in the Golgi apparatus (Tafesse et al. 2007; Villani et al. 2008; Ding et al. 2008). Phosphatidylcholine was identified as the source of the phosphocholine moiety donated to ceramide in this reaction in studies of the mouse enzyme in the 1970s (Diringer et al., 1972; Ullman and Radin, 1974). The association of SGMS2 with the plasma membrane appears to require palmitoylation of at least two cysteine residues near the carboxy terminus (Tani and Kuge, 2009). SGMS2 is widely expressed in the body, and while studies of cultured cells indicate that this is a minor source of cellular sphingomyelin, blockage of SGMS2 activity inhibits cell growth. SGMS2 deficiency causes forms of osteoporosis (CDL, MIM:126550; CDLSMD, MIM:126550) (Huitema et al., 2004; Tafesse et al., 2007; reviewed by Chen & Cao, 2017).

Literature References
PubMed ID Title Journal Year
28742512 The sphingomyelin synthase family: proteins, diseases, and inhibitors

Chen, Y, Cao, Y

Biol Chem 2017
4817756 The enzymatic formation of sphingomyelin from ceramide and lecithin in mouse liver

Radin, NS, Ullman, MD

J Biol Chem 1974
4339164 Evidence for a new biosynthetic pathway of sphingomyelin in SV 40 transformed mouse cells

Koch, MA, Diringer, H, Anderer, FA, Marggraf, WD

Biochem Biophys Res Commun 1972
17449912 Both sphingomyelin synthases SMS1 and SMS2 are required for sphingomyelin homeostasis and growth in human HeLa cells

van der Poel, S, Uphoff, A, Tafesse, FG, Huitema, K, Hermansson, M, Holthuis, JC, Somerharju, P, van den Dikkenberg, J

J Biol Chem 2007
19233134 Sphingomyelin synthase 2 is palmitoylated at the COOH-terminal tail, which is involved in its localization in plasma membranes

Tani, M, Kuge, O

Biochem Biophys Res Commun 2009
17982138 SMS overexpression and knockdown: impact on cellular sphingomyelin and diacylglycerol metabolism, and cell apoptosis

Hailemariam, T, Wu, MP, Ding, T, Maxfield, FR, Jiang, XC, Li, Z, Mukherjee, S

J Lipid Res 2008
14685263 Identification of a family of animal sphingomyelin synthases

Huitema, K, Brouwers, JF, Holthuis, JC, van den Dikkenberg, J

EMBO J 2004
Catalyst Activity

sphingomyelin synthase activity of 2xPalmC-SGMS2 [plasma membrane]

Orthologous Events
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