Biochemie und Pathobiochemie: Glycosyl-Phosphatidylinositol-Anker
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[Bearbeiten] Biosynthese eines Glycosyl-Phosphatidylinositol-Ankers (GPI-Anker)
| ⇓ | Subst. | ( ⇑ ) | Co. | Enzym | EC | EG | Erkr. |
|---|---|---|---|---|---|---|---|
1-Phosphatidyl-D-myo-Inositol
|
|||||||
| UDP-N-Acetyl-D-Glucosamin
UDP |
Phosphatidylinositol- N-Acetylglucosaminyltransferase | 2.4.1.198 | Tr | Paroxysmale nächtliche Hämoglobinurie (PNH) | |||
GlcNAcα1-6Ino1-P-Lipid
|
|||||||
| H2O | N-Acetylglucosaminyl- phosphatidylinositol-Deacetylase | 3.5.1.89 | Hyd | ||||
GlcNα1-6Ino1-P-Lipid
|
|||||||
| Palmitoyl-CoA
CoA-SH |
PIG-W | 2.3.-.- | Tr | ||||
GlcNα1-6Ino(palmitoyl)1-P-Lipid
|
|||||||
| Dolichyl-phosphat-D-Mannose
Dolichyl-phosphat |
PIG-M, PIG-X | 2.4.1.- | Tr | ||||
Manα1-4GlcNα1-6Ino(palmitoyl)1-P-Lipid
|
|||||||
| Phosphatidylethanolamin
Diacylglycerin |
PIG-N | 2.7.-.- | Tr | ||||
EtN-P
\
2
Manα1-4GlcNα1-6Ino(palmitoyl)1-P-Lipid
|
|||||||
| Dolichyl-phosphat-D-Mannose
Dolichyl-phosphat |
PIG-V | 2.4.1.- | Tr | ||||
EtN-P
\
2
Manα1-6Manα1-4GlcNα1-6Ino(palmitoyl)1-P-Lipid
|
|||||||
| Dolichyl-phosphat-D-Mannose
Dolichyl-phosphat |
PIG-B | 2.4.1.- | Tr | ||||
EtN-P
\
2
Manα1-2Manα1-6Manα1-4GlcNα1-6Ino(palmitoyl)1-P-Lipid
|
|||||||
| Dolichyl-phosphat-D-Mannose
Dolichyl-phosphat |
PIG-Z | 2.4.1.- | Tr | ||||
EtN-P
\
2
Manα1-2Manα1-2Manα1-6Manα1-4GlcNα1-6Ino(palmitoyl)1-P-Lipid
|
|||||||
| Phosphatidylethanolamin
Diacylglycerin |
PIG-F, PIG-O | ? | Tr | ||||
EtN-P EtN-P
\ \
6 2
Manα1-2Manα1-2Manα1-6Manα1-4GlcNα1-6Ino(palmitoyl)1-P-Lipid
|
|||||||
| Phosphatidylethanolamin
Diacylglycerin |
PIG-F, GPI-7 | ? | Tr | ||||
EtN-P EtN-P EtN-P
\ \ \
6 6 2
Manα1-2Manα1-2Manα1-6Manα1-4GlcNα1-6Ino(palmitoyl)1-P-Lipid
|
|||||||
| Protein
H2O |
GPAA1, PIG-K, PIG-S, PIG-T, PIG-U | ? | ? | ||||
H2N-Protein-CO-EtN-P EtN-P EtN-P
\ \ \
6 6 2
Manα1-2Manα1-2Manα1-6Manα1-4GlcNα1-6Ino(palmitoyl)1-P-Lipid
|
|||||||
| H2O
Fettsäure |
GPI-Deacylase | ? | ? | ||||
H2N-Protein-CO-EtN-P EtN-P EtN-P
\ \ \
6 6 2
Manα1-2Manα1-2Manα1-6Manα1-4GlcNα1-6Ino1-P-Lipid
|
|||||||
| Farbcode Monosaccharide: |
| Glc (D-Glucose) |
| Gal (D-Galactose) |
| Xyl (D-Xylulose) |
| Man (D-Mannose) |
| Fuc (D-Fucose) |
| GlcA (D-Glucuronat) |
| GalNAc (N-Acetyl-D-Galactosamin) |
| GlcNAc (N-Acetyl-D-Glucosamin) |
| GlcN (D-Glucosamin) |
| Neu5NAc (N-Acetylneuraminsäure) |
[Bearbeiten] Biologische Bedeutung
GPI-Anker dienen der Verankerung von Proteinen (z.B. Enzymen wie der Acetylcholinesterase) an der Zellmembran. GPI-Anker bestehen aus einem Phosphatidylinositol, wobei das Inositol ein Oligosaccharid trägt, an dem das Protein über ein Ethanolaminphosphat befestigt ist. Das Inositol kann zusätzlich mit einer weiteren Fettsäure (Palmitat) verestert sein.

