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 (SPT/LCB1-3) | A heterodimer membrane protein (LC1-LC2 or LC1-LC3) whose catalytic site faces the cytosol. In addition, small subunits (ssSPTa or ssSPTb) are essential for the full enzymatic activity.
| |
| LPTLC2 | |||
| LPTLC3 | |||
| ssSPTa, ssSPTb (SPTSSa/b) |
|||
| 3-ketodihydrosphingosine reductase | KDSR / FVT1 | ||
| Ceramide synthase | CerS1 (LAG1 longevity assurance homolog 1) |
19q12 | Brain, skeletal muscle, testis for C18 |
| CerS2 (Tumor metastasis-suppressor gene 1) |
1q21.2 | Kidney, liver and other >12 organs for C20-C26 | |
| CerS3 | 15q26.3 | Skin, testis for C22-C26 α-hydroxy chains | |
| CerS4 | 19q13.2 | Low in all tissues for C18-C22. Esp. skin, leucocytes, heart, liver. | |
| CerS5 | 12q13.3 | Low in all tissues for C16. Esp. muscle and brain. Major in lung epithelia. | |
| CerS6 | 2q24.3 | Low in all tissues for C14-C16 | |
| Dihydroceramide Δ4-desaturase | DEGS1 (DES1) | 1q42.11 | |
| DEGS2 (DES2) | The enzyme hydroxylates DES to form phytoceramide. | ||
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