Category:LBS/Biosynthesis/CeramideSyn

serine + palmitoyl (16:0)-CoA	serine palmitoyl transferase	3-ketodihydro- sphingosine (3-KDS)	3-KDS reductase	dihydrosphingosine (DHS, d18:0)	Cer synthase fatty acyl CoA	dihydroceramide (DHCer)	DHCer desaturase	ceramide (Cer)
Cytosolic surface of the endoplasmic reticulum (ER)

Enzyme function in the Human Genome
Protein name	Gene name (Uniprot)	Location
Serine palmitoyl transferase (SPT)	SPTLC1(LCB1)	9q22.31	A heterodimer membrane protein (LCB1-LCB2 or LCB1-LCB3) whose catalytic site faces the cytosol. In addition, small subunits (SPTSSA or SPTSSB) are essential for the full enzymatic activity. SPTSSA/B has no homology with the Tsc3p subunit in yeast.^[1] SPTLC1-SPTLC2-SPTSSA ... C16-CoA SPTLC1-SPTLC3-SPTSSA ... C14- and C16-CoA SPTLC1-SPTLC2-SPTSSB ... C18-CoA SPTLC1-SPTLC3-SPTSSB ... broad specificity
	SPTLC2 (LCB2,LCB2A)	14q24.3
	SPTLC3(LCB2B)	20p12.1
	SPTSSA(ssSPTa)	14q13.1
	SPTSSB(ssSPTb)	3q26.1
3-Ketodihydrosphingosine reductase	KDSR(FVT1)	18q21.33
Ceramide synthase ^[2]	CERS1 (LASS1,LAG1)	19p13.11	Brain, skeletal muscle, testis for C18
	CERS2 (LASS2,TMSG1)	1q21.3	Kidney, liver and other >12 organs for C20-C26
	CERS3(LASS3)	15q26.3	Skin, testis for C22-C26 α-hydroxy chains
	CERS4(LASS4)	19p13.2	Low in all tissues for C18-C22. Esp. skin, leucocytes, heart, liver.
	CERS5(LASS5)	12q13.12	Low in all tissues for C16. Esp. muscle and brain. Major in lung epithelia.
	CERS6(LASS6)	2q24.3	Low in all tissues for C14-C16
Dihydroceramide Δ4-desaturase	DEGS1(DES1)	1q42.11	The enzyme hydroxylates DES to form phytoceramide.^[3]
Dihydroceramide Δ4-desaturase	DEGS2(DES2)	14q32.2	The enzyme hydroxylates DES to form phytoceramide.^[3]
↑ Han G., Gupta S.D., Gable K., Niranjanakumari S., Moitra P., Eichler F., Brown R.H. Jr., Harmon J.M., Dunn T.M. (2009) "Identification of small subunits of mammalian serine palmitoyltransferase that confer distinct acyl-CoA substrate specificities" Proc. Natl. Acad. Sci. USA 106:8186-8191 PMID 19416851 ↑ Levy M, Futerman AH (2010) "Mammalian ceramide synthases" IUBMB Life 62(5):347-356 PMID 20222015 ↑ Michel C, van Echten-Deckert G. (1997) "Conversion of dihydroceramide to ceramide occurs at the cytosolic face of the endoplasmic reticulum" FEBS Lett 416(2):153-155 PMID 9369202

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[1] Han G., Gupta S.D., Gable K., Niranjanakumari S., Moitra P., Eichler F., Brown R.H. Jr., Harmon J.M., Dunn T.M. (2009) "Identification of small subunits of mammalian serine palmitoyltransferase that confer distinct acyl-CoA substrate specificities" Proc. Natl. Acad. Sci. USA 106:8186-8191 PMID 19416851

[2] Levy M, Futerman AH (2010) "Mammalian ceramide synthases" IUBMB Life 62(5):347-356 PMID 20222015

[3] Michel C, van Echten-Deckert G. (1997) "Conversion of dihydroceramide to ceramide occurs at the cytosolic face of the endoplasmic reticulum" FEBS Lett 416(2):153-155 PMID 9369202

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Category:LBS/Biosynthesis/CeramideSyn

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