| 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) | ||||||||
| [show]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]
|
| 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] |
| DEGS2(DES2) | 14q32.2 | ||
| |||
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