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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| [http://www.uniprot.org/uniprot/O15270 SPTLC2]
| [http://www.uniprot.org/uniprot/O15270 SPTLC2]<br/>(LCB2, LCB2A)
|  
| 14q24.3
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| [http://www.uniprot.org/uniprot/Q9NUV7 SPTLC3]
| [http://www.uniprot.org/uniprot/Q9NUV7 SPTLC3] (LCB2B)
|
| 20p12.1
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| [http://www.uniprot.org/uniprot/Q969W0 SPTSSA] (ssSPTa)
| [http://www.uniprot.org/uniprot/Q969W0 SPTSSA] (ssSPTa)
|
| 14q13.1
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|-
| [http://www.uniprot.org/uniprot/Q8NFR3 SPTSSB] (ssSPTb)
| [http://www.uniprot.org/uniprot/Q8NFR3 SPTSSB] (ssSPTb)
|
| 3q26.1
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|rowspan="1"| 3-Ketodihydrosphingosine reductase
|rowspan="1"| 3-Ketodihydrosphingosine reductase
| [http://www.uniprot.org/uniprot/Q06136 KDSR] (FVT1)
| [http://www.uniprot.org/uniprot/Q06136 KDSR] (FVT1)
|
| 18q21.33
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|rowspan="6"| Ceramide synthase
|rowspan="6"| Ceramide synthase
| CerS1<br/>(LAG1 longevity assurance homolog 1)
| [http://www.uniprot.org/uniprot/P27544 CERS1]<br/>(LASS1, LAG1)
| 19q12
| 19p13.11
| Brain, skeletal muscle, testis for C18
| Brain, skeletal muscle, testis for C18
|-
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| [http://www.uniprot.org/uniprot/Q96G23 CerS2]<br/>(Tumor metastasis-suppressor gene 1)
| [http://www.uniprot.org/uniprot/Q96G23 CERS2]<br/>(LASS2, TMSG1)
| 1q21.2
| 1q21.3
| Kidney, liver and other >12 organs for C20-C26
| Kidney, liver and other >12 organs for C20-C26
|-
|-
| CerS3
| [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
|-
|-
| CerS4
| [http://www.uniprot.org/uniprot/Q9HA82 CERS4] (LASS4)
| 19q13.2
| 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.
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| CerS5
| [http://www.uniprot.org/uniprot/Q8N5B7 CERS5] (LASS5)
| 12q13.3
| 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.
|-
|-
| CerS6
| [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
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|rowspan="2"|Dihydroceramide &Delta;4-desaturase  
|rowspan="2"|Dihydroceramide &Delta;4-desaturase  
| DEGS1 (DES1)
| [http://www.uniprot.org/uniprot/O15121 DEGS1] (DES1)
| 1q42.11
| 1q42.11
|
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|-
| DEGS2 (DES2)
| [http://www.uniprot.org/uniprot/Q6QHC5 DEGS2] (DES2)
|
| 14q32.2
| The enzyme hydroxylates DES to form phytoceramide.
| The enzyme hydroxylates DES to form phytoceramide.
|}
|}

Revision as of 03:32, 18 December 2017

serine
+
palmitoyl (16:0)-CoA

Arrow00r.png
 serine palmitoyl
  transferase
3-ketodihydro-
  sphingosine
(3-KDS)

Arrow00r.png
  3-KDS
  reductase
dihydrosphingosine
(DHS, d18:0)
Cer synthase
Arrow1rr.png
fatty acyl CoA
dihydroceramide
(DHCer)

Arrow00r.png
  DHCer
  desaturase
ceramide
(Cer)
Cytosolic surface of the endoplasmic reticulum (ER)

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