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 ( | |rowspan="5"| 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.<ref>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</ref> | ||
<ul> | <ul> | ||
<li>SPTLC1-SPTLC2-SPTSSA ... C16-CoA | <li>SPTLC1-SPTLC2-SPTSSA ... C16-CoA | ||
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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 | ||
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|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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|rowspan="6"| Ceramide synthase | |rowspan="6"| Ceramide synthase <ref>Levy M, Futerman AH (2010) "Mammalian ceramide synthases" IUBMB Life 62(5):347-356 PMID 20222015</ref> | ||
| [http://www.uniprot.org/uniprot/P27544 CERS1]<br/>(LASS1,LAG1) | | [http://www.uniprot.org/uniprot/P27544 CERS1]<br/>(LASS1,LAG1) | ||
| 19p13.11 | | 19p13.11 | ||
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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 | ||
| | |rowspan="2"| The enzyme hydroxylates DES to form phytoceramide.<ref>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</ref> | ||
|- | |- | ||
| [http://www.uniprot.org/uniprot/Q6QHC5 DEGS2] (DES2) | | [http://www.uniprot.org/uniprot/Q6QHC5 DEGS2](DES2) | ||
| 14q32.2 | | 14q32.2 | ||
| | |- | ||
|colspan="4"|<references/> | |||
|} | |} | ||
Latest revision as of 04:30, 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 (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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