Biochemie und Pathobiochemie: Abbau der Glycosaminoglycane
Abbau von Dermatansulfat (Chondroitinsulfat B)
[Bearbeiten]| ⇓ | Subst. | ( ⇑ ) | Co. | Enzym | EC | EG | Erkr. |
|---|---|---|---|---|---|---|---|
|
R-[4IdoA(2S)β1-3GalNAc(4S)β1-4GlcAβ1-3GalNAc(4S)β1]n-R Dermatansulfat (Chondroitinsulfat B) |
|||||||
| H2O
Dermatansulfat |
|
3.2.-.- | Hyd | ||||
|
IdoA(2S)β1-3GalNAc(4S)β1-4GlcAβ1-3GalNAc(4S) |
|||||||
| H2O |
|
Iduronat-2-Sulfatase | 3.1.6.13 | Hyd | Mucopolysaccharidose II (Hunter) | ||
|
IdoAβ1-3GalNAc(4S)β1-4GlcAβ1-3GalNAc(4S) |
|||||||
| H2O
L-Iduronat |
|
L-Iduronidase | 3.2.1.76 | Hyd | Mucopolysaccharidose IH (Hurler), IS (Scheie) und IH/S (Hurler/Scheie) | ||
|
GalNAc(4S)β1-4GlcAβ1-3GalNAc(4S) |
|||||||
| H2O |
|
Arylsulfatase B | 3.1.6.12 | Hyd | Mucopolysaccharidose VI (Maroteaux-Lamy) | ||
|
GalNAcβ1-4GlcAβ1-3GalNAc(4S) |
|||||||
| H2O |
|
Hyalurono- glucosaminidase | 3.2.1.35 | Hyd | Mucopolysaccharidose IX | ||
|
GlcAβ1-3GalNAc(4S) |
|||||||
N-Acetylgalactosamin-4-sulfat kann auch direkt abgespalten werden:
| ⇓ | Subst. | ( ⇑ ) | Co. | Enzym | EC | EG | Erkr. |
|---|---|---|---|---|---|---|---|
|
GalNAc(4S)β1-4GlcAβ1-3GalNAc(4S) |
|||||||
| H2O
N-Acetylgalactosamin-4-sulfat |
|
Hyalurono- glucosaminidase | 3.2.1.35 | Hyd | Mucopolysaccharidose IX | ||
|
GlcAβ1-3GalNAc(4S) |
|||||||
Abbau von Chondroitinsulfat
[Bearbeiten]Der Abbau entspricht den letzten Schritten des Abbaus von Dermatansulfat.
| ⇓ | Subst. | ( ⇑ ) | Co. | Enzym | EC | EG | Erkr. |
|---|---|---|---|---|---|---|---|
|
R-[3GalNAc(S)β1-4GlcAβ1-3GalNAc(S)β1]-R Chondroitinsulfat |
|||||||
| H2O
Chondroitinsulfat |
|
3.2.-.- | Hyd | ||||
|
GalNAc(S)β1-4GlcAβ1-3GalNAc(S) |
|||||||
| H2O |
|
Arylsulfatase B | 3.1.6.12 | Hyd | Mucopolysaccharidose VI (Maroteaux-Lamy) | ||
|
GalNAcβ1-4GlcAβ1-3GalNAc(S) |
|||||||
| H2O |
|
Hyalurono- glucosaminidase | 3.2.1.35 | Hyd | Mucopolysaccharidose IX | ||
|
GlcAβ1-3GalNAc(S) |
|||||||
N-Acetylgalactosamin-4-sulfat kann auch hier direkt abgespalten werden:
| ⇓ | Subst. | ( ⇑ ) | Co. | Enzym | EC | EG | Erkr. |
|---|---|---|---|---|---|---|---|
|
GalNAc(S)β1-4GlcAβ1-3GalNAc(S) |
|||||||
| H2O
N-Acetylgalactosamin-4-sulfat |
|
Hyalurono- glucosaminidase | 3.2.1.35 | Hyd | Mucopolysaccharidose IX | ||
|
GlcAβ1-3GalNAc(S) |
|||||||
Abbau von Heparansulfat
[Bearbeiten]| ⇓ | Subst. | ( ⇑ ) | Co. | Enzym | EC | EG | Erkr. |
|---|---|---|---|---|---|---|---|
|
R-[4IdoA(2S)β1-4GlcN(2S)α1-4GlcA(2S)β1-4GlcNAc(6S)α1-4GlcAβ1]-R Heparansulfat |
|||||||
| H2O
Heparansulfat |
|
HPSE, HPSE2 | 3.2.-.- | Hyd | |||
|
IdoA(2S)β1-4GlcN(2S)α1-4GlcA(2S)β1-4GlcNAc(6S)α1-4GlcA |
|||||||
| H2O |
|
Iduronat-2-sulfatase | 3.1.6.13 | Hyd | Mucopoly- saccharidose II (Hunter) | ||
|
IdoAβ1-4GlcN(2S)α1-4GlcA(2S)β1-4GlcNAc(6S)α1-4GlcA |
|||||||
| H2O
L-Iduronat |
|
L-Iduronidase | 3.2.1.76 | Hyd | Mucopolysaccharidose IH (Hurler), IS (Scheie) und IH/S (Hurler/Scheie) | ||
|
GlcN(2S)α1-4GlcA(2S)β1-4GlcNAc(6S)α1-4GlcA |
|||||||
| H2O |
|
N-Sulfoglucosamin- Sulfohydrolase (Sulfamidase) | 3.10.1.1 | Hyd | Mucopoly- saccharidose IIIA (Sanfilippo A) | ||
|
GlcNα1-4GlcA(2S)β1-4GlcNAc(6S)α1-4GlcA |
|||||||
| Acetyl-CoA
CoA-SH |
|
Heparan- α-Glucosaminid- N-Acetyltransferase | 2.3.1.78 | Tr | Mucopoly- saccharidose IIIC (Sanfilippo C) | ||
|
GlcNAcα1-4GlcA(2S)β1-4GlcNAc(6S)α1-4GlcA |
|||||||
| H2O |
|
α-N-Acetyl- glucosaminidase | 3.2.1.50 | Hyd | Mucopoly- saccharidose IIIB (Sanfilippo B) | ||
|
GlcA(2S)β1-4GlcNAc(6S)α1-4GlcA |
|||||||
| H2O |
|
Glucuronat- 2-Sulfatase | 3.1.6.18 | Hyd | |||
|
GlcAβ1-4GlcNAc(6S)α1-4GlcA |
|||||||
| H2O |
|
β-Glucuronidase | 3.2.1.31 | Hyd | Mucopoly- saccharidose VII | ||
|
GlcNAc(6S)α1-4GlcA |
|||||||
| H2O |
|
N-Acetylglucosamin- 6-sulfatase | 3.1.6.14 | Hyd | Mucopoly- saccharidose IIID (Sanfilippo D) | ||
|
GlcNAcα1-4GlcA |
|||||||
Abbau von Keratansulfat
[Bearbeiten]| ⇓ | Subst. | ( ⇑ ) | Co. | Enzym | EC | EG | Erkr. |
|---|---|---|---|---|---|---|---|
|
R-[3Gal(6S)β1-4GlcNAc(6S)β1-3Galβ1-4GlcNAc(6S)β1-3Galβ1]-R |
|||||||
| H2O
Keratansulfat |
|
Keratansulfat-Endo- 1,4-β-galactosidase | 3.2.1.103 | Hyd | |||
|
Gal(6S)β1-4GlcNAc(6S)β1-3Galβ1-4GlcNAc(6S)β1-3Gal |
|||||||
| H2O |
|
N-Acetylgalactosamin- 6-sulfatase | 3.1.6.4 | Hyd | Mucopoly- saccharidose IVA (Morquio A) | ||
|
Galβ1-4GlcNAc(6S)β1-3Galβ1-4GlcNAc(6S)β1-3Gal |
|||||||
| H2O |
|
β-Galactosidase | 3.2.1.23 | Hyd | GM1-Gangliosidose, Mucopolysaccharidose IVB (Morquio B) | ||
|
GlcNAc(6S)β1-3Galβ1-4GlcNAc(6S)β1-3Gal |
|||||||
| H2O |
|
N-Acetylglucosamin- 6-sulfatase | 3.1.6.14 | Hyd | Mucopoly- saccharidose IIID (Sanfilippo D) | ||
|
GlcNAcβ1-3Galβ1-4GlcNAc(6S)β1-3Gal |
|||||||
| H2O |
|
β-N-Acetyl- hexosaminidase | 3.2.1.52 | Hyd | GM2-Gangliosidose I (Tay-Sachs), II (Sandhoff) | ||
|
Galβ1-4GlcNAc(6S)β1-3Gal |
|||||||
N-Acetyl-D-Glucosamin-6-sulfat kann auch direkt abgespalten werden:
| ⇓ | Subst. | ( ⇑ ) | Co. | Enzym | EC | EG | Erkr. |
|---|---|---|---|---|---|---|---|
|
GlcNAc(6S)β1-3Galβ1-4GlcNAc(6S)β1-3Gal |
|||||||
| H2O
N-Acetyl-D-Glucosamin-6-sulfat |
|
β-N-Acetylhexosaminidase | 3.2.1.52 | Hyd | GM2-Gangliosidose I (Tay-Sachs), II (Sandhoff) | ||
|
Galβ1-4GlcNAc(6S)β1-3Gal |
|||||||
| Farbcode Monosaccharide: |
| Glc (D-Glucose) |
| Gal (D-Galactose) |
| Xyl (D-Xylose) |
| Man (D-Mannose) |
| Fuc (L-Fucose) |
| GlcA (D-Glucuronat) |
| IdoA (L-Iduronat) |
| GalNAc (N-Acetyl-D-Galactosamin) |
| GlcNAc (N-Acetyl-D-Glucosamin) |
| GlcN (D-Glucosamin) |
| Neu5NAc (N-Acetylneuraminsäure) |
Weblinks
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