Category:LBS/Biosynthesis/CeramideSyn: Difference between revisions
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|style="background:darkseagreen;border-radius: 20px 0px 0px 0px" | serine<br/>+<br/>palmitoyl (16:0)-CoA | |style="background:darkseagreen;border-radius: 20px 0px 0px 0px" | serine<br/>+<br/>palmitoyl (16:0)-CoA | ||
|style="background:darkseagreen" | <br/>[[File:Arrow00r.png]]<br/> <small>serine palmitoyl | |style="background:darkseagreen" | <br/>[[File:Arrow00r.png]]<br/> <small>serine palmitoyl<br/> transferase</small> | ||
|style="background:darkseagreen" | 3- | |style="background:darkseagreen" | 3-ketodihydro-<br/> sphingosine<br/>(3-KDS) | ||
|style="background:darkseagreen" | <br/>[[File:Arrow00r.png]]<br/> <small>3- | |style="background:darkseagreen" | <br/>[[File:Arrow00r.png]]<br/> <small>3-KDS<br/> reductase</small> | ||
|style="background:darkseagreen" | | |style="background:darkseagreen" | dihydrosphingosine<br/>(DHS, d18:0) | ||
|style="background:darkseagreen" | <small> | |style="background:darkseagreen" | <small>Cer synthase</small><br/>[[File:Arrow1rr.png]]<br/><small>fatty acyl CoA</small> | ||
|style="background:darkseagreen" | dihydroceramide<br/>(DHCer) | |style="background:darkseagreen" | dihydroceramide<br/>(DHCer) | ||
|style="background:darkseagreen" | <br/>[[File:Arrow00r.png]]<br/> <small> | |style="background:darkseagreen" | <br/>[[File:Arrow00r.png]]<br/> <small>DHCer<br/> desaturase</small> | ||
|style="background:darkseagreen;border-radius: 0px 20px 0px 0px" | ceramide<br/>(Cer) | |style="background:darkseagreen;border-radius: 0px 20px 0px 0px" | ceramide<br/>(Cer) | ||
|- | |- | ||
|colspan="9" style="background:darkseagreen;border-radius: 0px 0px 20px 20px; font-weight: 600; text-shadow:black 3px 3px 5px; color: white;"| <big> | |colspan="9" style="background:darkseagreen;border-radius: 0px 0px 20px 20px; font-weight: 600; text-shadow:black 3px 3px 5px; color: white;"| <big>Cytosolic surface of the endoplasmic reticulum (ER)</big> | ||
|} | |||
{| class="wikitable collapsible collapsed" style="width: 100%" | |||
!colspan="4"| Enzyme function in the Human Genome | |||
|- | |||
! Name | |||
! Human gene | |||
! Location | |||
! | |||
|- | |||
|rowspan="4"| serine palmitoyl transferase (SPT) | |||
| [http://www.uniprot.org/uniprot/O15269 SPTLC1] (SPT/LCB1-3) | |||
| | |||
|rowspan="4"| 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. | |||
<ul> | |||
<li>SPTLC1-SPTLC2-ssSPTa ... C16-CoA | |||
<li>SPTLC1-SPTLC3-ssSPTa ... C14- and C16-CoA | |||
<li>SPTLC1-SPTLC2-ssSPTb ... C18-CoA | |||
<li>SPTLC1-SPTLC3-ssSPTb ... broad specificity | |||
</ul> | |||
|- | |||
| LPTLC2 | |||
| | |||
|- | |||
| LPTLC3 | |||
| | |||
|- | |||
| ssSPTa, ssSPTb <br/> (SPTSSa/b) | |||
| | |||
|- | |||
| 3-ketodihydrosphingosine reductase | |||
| KDSR / FVT1 | |||
| | |||
| | |||
|- | |||
|rowspan="6"| ceramide synthase | |||
| CerS1<br/>(LAG1 longevity assurance homolog 1) | |||
| 19q12 | |||
| Brain, skeletal muscle, testis for C18 | |||
|- | |||
| [http://www.uniprot.org/uniprot/Q96G23 CerS2]<br/>(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 | |||
|- | |||
|rowspan="2"|dihydroceramide Δ4-desaturase | |||
| DES1 | |||
| | |||
| | |||
|- | |||
| DES2 | |||
| | |||
| The enzyme hydroxylates DES to form phytoceramide. | |||
|} | |} | ||
Revision as of 22:35, 17 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 | |||
|---|---|---|---|
| Name | Human gene | 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 | DES1 | ||
| DES2 | The enzyme hydroxylates DES to form phytoceramide. | ||
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