|
|
| Line 46: |
Line 46: |
| |- | | |- |
| |rowspan="1"| 3-Ketodihydrosphingosine reductase | | |rowspan="1"| 3-Ketodihydrosphingosine reductase |
| |-
| |
| | [http://www.uniprot.org/uniprot/Q06136 KDSR] | | | [http://www.uniprot.org/uniprot/Q06136 KDSR] |
| |
| |
| |- | | |- |
| |rowspan="6"| Ceramide synthase | | |rowspan="6"| Ceramide synthase |
Revision as of 10:55, 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)
|
| [show]Enzyme function in the Human Genome
|
| Protein name
|
Gene name (Uniprot)
|
Location
|
|
| Serine palmitoyl transferase (SPT)
|
SPTLC1
|
|
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
|
| SPTLC2
|
|
| SPTLC3
|
|
| SPTSSA (ssSPTa)
|
|
| SPTSSB (ssSPTb)
|
|
| 3-Ketodihydrosphingosine reductase
|
KDSR
|
| 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
|
DEGS1 (DES1)
|
1q42.11
|
|
| DEGS2 (DES2)
|
|
The enzyme hydroxylates DES to form phytoceramide.
|
This category currently contains no pages or media.
