Category:LBS/Biosynthesis/CeramideSyn: Difference between revisions
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|rowspan="5"| Serine palmitoyl transferase (SPT) | |rowspan="5"| Serine palmitoyl transferase (SPT) | ||
| [http://www.uniprot.org/uniprot/O15269 SPTLC1] (LCB1) | | [http://www.uniprot.org/uniprot/O15269 SPTLC1](LCB1) | ||
| 9q22.31 | | 9q22.31 | ||
|rowspan="5"| A heterodimer membrane protein (LC1-LC2 or LC1-LC3) whose catalytic site faces the cytosol. In addition, small subunits (SPTSSA or SPTSSB) are essential for the full enzymatic activity. | |rowspan="5"| A heterodimer membrane protein (LC1-LC2 or LC1-LC3) whose catalytic site faces the cytosol. In addition, small subunits (SPTSSA or SPTSSB) are essential for the full enzymatic activity. | ||
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| 14q24.3 | | 14q24.3 | ||
|- | |- | ||
| [http://www.uniprot.org/uniprot/Q9NUV7 SPTLC3] (LCB2B) | | [http://www.uniprot.org/uniprot/Q9NUV7 SPTLC3](LCB2B) | ||
| 20p12.1 | | 20p12.1 | ||
|- | |- | ||
| [http://www.uniprot.org/uniprot/Q969W0 SPTSSA] (ssSPTa) | | [http://www.uniprot.org/uniprot/Q969W0 SPTSSA](ssSPTa) | ||
| 14q13.1 | | 14q13.1 | ||
|- | |- | ||
| [http://www.uniprot.org/uniprot/Q8NFR3 SPTSSB] (ssSPTb) | | [http://www.uniprot.org/uniprot/Q8NFR3 SPTSSB](ssSPTb) | ||
| 3q26.1 | | 3q26.1 | ||
|- | |- | ||
|rowspan="1"| 3-Ketodihydrosphingosine reductase | |rowspan="1"| 3-Ketodihydrosphingosine reductase | ||
| [http://www.uniprot.org/uniprot/Q06136 KDSR] | | [http://www.uniprot.org/uniprot/Q06136 KDSR](FVT1) | ||
| 18q21.33 | | 18q21.33 | ||
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| Kidney, liver and other >12 organs for C20-C26 | | Kidney, liver and other >12 organs for C20-C26 | ||
|- | |- | ||
| [http://www.uniprot.org/uniprot/Q8IU89 CERS3] (LASS3) | | [http://www.uniprot.org/uniprot/Q8IU89 CERS3](LASS3) | ||
| 15q26.3 | | 15q26.3 | ||
| Skin, testis for C22-C26 α-hydroxy chains | | Skin, testis for C22-C26 α-hydroxy chains | ||
|- | |- | ||
| [http://www.uniprot.org/uniprot/Q9HA82 CERS4] (LASS4) | | [http://www.uniprot.org/uniprot/Q9HA82 CERS4](LASS4) | ||
| 19p13.2 | | 19p13.2 | ||
| Low in all tissues for C18-C22. Esp. skin, leucocytes, heart, liver. | | Low in all tissues for C18-C22. Esp. skin, leucocytes, heart, liver. | ||
|- | |- | ||
| [http://www.uniprot.org/uniprot/Q8N5B7 CERS5] (LASS5) | | [http://www.uniprot.org/uniprot/Q8N5B7 CERS5](LASS5) | ||
| 12q13.12 | | 12q13.12 | ||
| Low in all tissues for C16. Esp. muscle and brain. Major in lung epithelia. | | Low in all tissues for C16. Esp. muscle and brain. Major in lung epithelia. | ||
|- | |- | ||
| [http://www.uniprot.org/uniprot/Q6ZMG9 CERS6] (LASS6) | | [http://www.uniprot.org/uniprot/Q6ZMG9 CERS6](LASS6) | ||
| 2q24.3 | | 2q24.3 | ||
| Low in all tissues for C14-C16 | | Low in all tissues for C14-C16 | ||
|- | |- | ||
|rowspan="2"|Dihydroceramide Δ4-desaturase | |rowspan="2"|Dihydroceramide Δ4-desaturase | ||
| [http://www.uniprot.org/uniprot/O15121 DEGS1] (DES1) | | [http://www.uniprot.org/uniprot/O15121 DEGS1](DES1) | ||
| 1q42.11 | | 1q42.11 | ||
| | | | ||
|- | |- | ||
| [http://www.uniprot.org/uniprot/Q6QHC5 DEGS2] (DES2) | | [http://www.uniprot.org/uniprot/Q6QHC5 DEGS2](DES2) | ||
| 14q32.2 | | 14q32.2 | ||
| The enzyme hydroxylates DES to form phytoceramide. | | The enzyme hydroxylates DES to form phytoceramide. | ||
|} | |} | ||
Revision as of 03:42, 18 December 2017
| 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 (LC1-LC2 or LC1-LC3) whose catalytic site faces the cytosol. In addition, small subunits (SPTSSA or SPTSSB) are essential for the full enzymatic activity.
|
| 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 | 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 | |
| DEGS2(DES2) | 14q32.2 | The enzyme hydroxylates DES to form phytoceramide. | |
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