Enzymes
| UniProtKB help_outline | 612 proteins |
Reaction participants Show >> << Hide
- Name help_outline a globoside IV3GalNAc-Gb4Cer Identifier CHEBI:90400 Charge 0 Formula C38H63N3O28R2 SMILEShelp_outline [C@@H]1([C@H](O[C@@H](O[C@@H]2[C@H]([C@H](O[C@H]3[C@H](O[C@@H](O[C@@H]4[C@H](O[C@@H](OC[C@@H]([C@@H](*)O)NC(=O)*)[C@@H]([C@H]4O)O)CO)[C@@H]([C@H]3O)O)CO)O[C@@H]([C@@H]2O)CO)O)[C@@H]([C@H]1O[C@H]5[C@@H]([C@H]([C@@H](O)[C@H](O5)CO)O)NC(C)=O)NC(C)=O)CO)O 2D coordinates Mol file for the small molecule Search links Involved in 3 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
- Name help_outline H2O Identifier CHEBI:15377 (Beilstein: 3587155; CAS: 7732-18-5) help_outline Charge 0 Formula H2O InChIKeyhelp_outline XLYOFNOQVPJJNP-UHFFFAOYSA-N SMILEShelp_outline [H]O[H] 2D coordinates Mol file for the small molecule Search links Involved in 6,048 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
- Name help_outline a globoside Gb4Cer Identifier CHEBI:88167 Charge 0 Formula C30H50N2O23R2 SMILEShelp_outline [C@H]([C@@H](*)O)(NC(=O)*)CO[C@@H]1O[C@@H]([C@@H](O[C@@H]2O[C@@H]([C@H](O[C@@H]3[C@@H]([C@@H](O[C@H]4[C@@H]([C@H]([C@@H](O)[C@H](O4)CO)O)NC(C)=O)[C@H]([C@@H](CO)O3)O)O)[C@@H]([C@H]2O)O)CO)[C@@H]([C@H]1O)O)CO 2D coordinates Mol file for the small molecule Search links Involved in 10 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
- Name help_outline N-acetyl-α-D-galactosamine Identifier CHEBI:40356 (CAS: 14215-68-0) help_outline Charge 0 Formula C8H15NO6 InChIKeyhelp_outline OVRNDRQMDRJTHS-CBQIKETKSA-N SMILEShelp_outline CC(=O)N[C@H]1[C@@H](O)O[C@H](CO)[C@H](O)[C@@H]1O 2D coordinates Mol file for the small molecule Search links Involved in 4 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
Cross-references
| RHEA:48412 | RHEA:48413 | RHEA:48414 | RHEA:48415 | |
|---|---|---|---|---|
| Reaction direction help_outline | undefined | left-to-right | right-to-left | bidirectional |
| UniProtKB help_outline |
|
Publications
-
An investigation into the glycolipid metabolism of alpha-N-acetylgalactosaminidase-deficient fibroblasts using native and artificial glycolipids.
Klima B., Pohlentz G., Schindler D., Egge H.
Deficient activity of lysosomal alpha-N-acetylgalactosaminidase represents a recently recognized lysosomal disorder whose neurologic manifestation in infancy is infantile neuroaxonal dystrophy. The lysosomal enzyme defect, inherited as an autosomal recessive trait, was first identified in the two ... >> More
Deficient activity of lysosomal alpha-N-acetylgalactosaminidase represents a recently recognized lysosomal disorder whose neurologic manifestation in infancy is infantile neuroaxonal dystrophy. The lysosomal enzyme defect, inherited as an autosomal recessive trait, was first identified in the two brothers, GD and BD. Metabolic modification of glycolipids with terminal alpha-GalNAc was studied in fibroblasts from these patients. [Ceramide-3H]Forssman-glycosphingolipid (GSL), the fluorescent C6-NBD-lyso-Forssman-glycolipid (GL) and a 14C-labelled neoglycolipid containing the blood group A trisaccharide were synthesized and used as probes in degradation studies with cell homogenates and with cells in culture. Assays of each of these substrates with fibroblast homogenates of the patients demonstrated the profound deficiency of alpha-N-acetylgalactosaminidase activity compared with controls. Residual activities in the patients' fibroblast homogenates were detected with all glycolipid substrates; those amounted to 6.3 +/- 3.7% (BD) and 12.8 +/-6.3% (GD) of the mean activity in controls for [3H]Forssman-GSL, and to 2.2 +/-0.8% (BD) and 3.6 +/-1.8% (GD) for C6-NBD-lyso-Forssman GL, respectively. alpha-N-Acetylgalactosaminidase deficiency in intact cells was confirmed by TLC analyses, which showed impaired glycolipid modification in cell extracts obtained following addition of [3H]Forssman GSL and C6-NBD-lyso-Forssman GL to the culture media of fibroblasts from the patients. << Less
Biol. Chem. Hoppe-Seyler 373:989-999(1992) [PubMed] [EuropePMC]