Reactome | SGMS2 transfers phosphocholine onto ceramide

SGMS2 transfers phosphocholine onto ceramide

Stable Identifier

R-HSA-429786

Type

Reaction [transition]

Species

Homo sapiens

Compartment

plasma membrane

Synonyms

phosphatidylcholine + ceramide <=> sphingomyelin + diacylglycerol [SGMS2]

ReviewStatus

5/5

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SGMS2 transfers phosphocholine onto ceramide

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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 Ullman, MD, Radin, NS	J Biol Chem	1974
4339164	Evidence for a new biosynthetic pathway of sphingomyelin in SV 40 transformed mouse cells Diringer, H, Marggraf, WD, Koch, MA, Anderer, FA	Biochem Biophys Res Commun	1972
17449912	Both sphingomyelin synthases SMS1 and SMS2 are required for sphingomyelin homeostasis and growth in human HeLa cells Tafesse, FG, Huitema, K, Hermansson, M, van der Poel, S, van den Dikkenberg, J, Uphoff, A, Somerharju, P, Holthuis, JC	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 Ding, T, Li, Z, Hailemariam, T, Mukherjee, S, Maxfield, FR, Wu, MP, Jiang, XC	J Lipid Res	2008
14685263	Identification of a family of animal sphingomyelin synthases Huitema, K, van den Dikkenberg, J, Brouwers, JF, Holthuis, JC	EMBO J	2004