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| |rowspan="6"| Ceramide synthase | | |rowspan="6"| Ceramide synthase |
| | [http://www.uniprot.org/uniprot/P27544 CERS1]<br/>(LASS1, LAG1) | | | [http://www.uniprot.org/uniprot/P27544 CERS1]<br/>(LASS1,LAG1) |
| | 19p13.11 | | | 19p13.11 |
| | Brain, skeletal muscle, testis for C18 | | | Brain, skeletal muscle, testis for C18 |
| |- | | |- |
| | [http://www.uniprot.org/uniprot/Q96G23 CERS2]<br/>(LASS2, TMSG1) | | | [http://www.uniprot.org/uniprot/Q96G23 CERS2]<br/>(LASS2,TMSG1) |
| | 1q21.3 | | | 1q21.3 |
| | Kidney, liver and other >12 organs for C20-C26 | | | Kidney, liver and other >12 organs for C20-C26 |
Revision as of 12:33, 18 December 2017
serine + palmitoyl (16:0)-CoA
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 serine palmitoyl transferase
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3-ketodihydro- sphingosine (3-KDS)
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 3-KDS reductase
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dihydrosphingosine (DHS, d18:0)
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Cer synthase
 fatty acyl CoA
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dihydroceramide (DHCer)
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 DHCer desaturase
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ceramide (Cer)
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Cytosolic surface of the endoplasmic reticulum (ER)
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[show]Enzyme function in the Human Genome
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Protein name
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Gene name (Uniprot)
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Location
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Serine palmitoyl transferase (SPT)
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SPTLC1 (LCB1)
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9q22.31
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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.
- SPTLC1-SPTLC2-SPTSSA ... C16-CoA
- SPTLC1-SPTLC3-SPTSSA ... C14- and C16-CoA
- SPTLC1-SPTLC2-SPTSSB ... C18-CoA
- SPTLC1-SPTLC3-SPTSSB ... broad specificity
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SPTLC2 (LCB2, LCB2A)
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14q24.3
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SPTLC3 (LCB2B)
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20p12.1
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SPTSSA (ssSPTa)
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14q13.1
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SPTSSB (ssSPTb)
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3q26.1
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3-Ketodihydrosphingosine reductase
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KDSR (FVT1)
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18q21.33
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Ceramide synthase
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CERS1 (LASS1,LAG1)
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19p13.11
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Brain, skeletal muscle, testis for C18
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CERS2 (LASS2,TMSG1)
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1q21.3
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Kidney, liver and other >12 organs for C20-C26
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CERS3 (LASS3)
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15q26.3
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Skin, testis for C22-C26 α-hydroxy chains
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CERS4 (LASS4)
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19p13.2
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Low in all tissues for C18-C22. Esp. skin, leucocytes, heart, liver.
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CERS5 (LASS5)
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12q13.12
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Low in all tissues for C16. Esp. muscle and brain. Major in lung epithelia.
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CERS6 (LASS6)
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2q24.3
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Low in all tissues for C14-C16
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Dihydroceramide Δ4-desaturase
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DEGS1 (DES1)
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1q42.11
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DEGS2 (DES2)
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14q32.2
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The enzyme hydroxylates DES to form phytoceramide.
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