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- 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 N-acetyl-β-D-6-sulfogalactosaminyl-(1→4)-α-L-iduronyl-(1→3)-N-acetyl-D-6-sulfogalactosamine Identifier CHEBI:152567 Charge -3 Formula C22H33N2O23S2 InChIKeyhelp_outline JYUWZQTYAWSZTE-CBMSEYHFSA-K SMILEShelp_outline [C@@H]1([C@H]([C@@H]([C@H]([C@@H](O1)O[C@@H]2[C@H](C(O[C@@H]([C@@H]2O)COS([O-])(=O)=O)O)NC(C)=O)O)O)O[C@H]3[C@@H]([C@H]([C@H]([C@H](O3)COS([O-])(=O)=O)O)O)NC(C)=O)C([O-])=O 2D coordinates Mol file for the small molecule Search links Involved in 1 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
- Name help_outline α-L-iduronyl-(1→3)-N-acetyl-D-6-sulfogalactosamine Identifier CHEBI:152568 Charge -2 Formula C14H21NO15S InChIKeyhelp_outline HMCUNCSRFXXUOR-DAXMSAKASA-L SMILEShelp_outline [C@@H]1([C@H]([C@@H]([C@H]([C@@H](O1)O[C@@H]2[C@H](C(O[C@@H]([C@@H]2O)COS([O-])(=O)=O)O)NC(C)=O)O)O)O)C([O-])=O 2D coordinates Mol file for the small molecule Search links Involved in 1 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
- Name help_outline N-acetyl-D-6-sulfogalactosamine Identifier CHEBI:153064 Charge -1 Formula C8H14NO9S InChIKeyhelp_outline WJFVEEAIYIOATH-KEWYIRBNSA-M SMILEShelp_outline O[C@@H]1[C@H](C(O[C@@H]([C@@H]1O)COS([O-])(=O)=O)O)NC(C)=O 2D coordinates Mol file for the small molecule Search links Involved in 1 reaction(s) Find molecules that contain or resemble this structure Find proteins in UniProtKB for this molecule
Cross-references
| RHEA:64384 | RHEA:64385 | RHEA:64386 | RHEA:64387 | |
|---|---|---|---|---|
| Reaction direction help_outline | undefined | left-to-right | right-to-left | bidirectional |
| UniProtKB help_outline |
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Publications
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Physiological substrates for human lysosomal beta -hexosaminidase S.
Hepbildikler S.T., Sandhoff R., Kolzer M., Proia R.L., Sandhoff K.
Human lysosomal beta-hexosaminidases remove terminal beta-glycosidically bound N-acetylhexosamine residues from a number of glycoconjugates. Three different isozymes composed of two noncovalently linked subunits alpha and beta exist: Hex A (alphabeta), Hex B (betabeta), and Hex S (alphaalpha). Whi ... >> More
Human lysosomal beta-hexosaminidases remove terminal beta-glycosidically bound N-acetylhexosamine residues from a number of glycoconjugates. Three different isozymes composed of two noncovalently linked subunits alpha and beta exist: Hex A (alphabeta), Hex B (betabeta), and Hex S (alphaalpha). While the role of Hex A and B for the degradation of several anionic and neutral glycoconjugates has been well established, the physiological significance of labile Hex S has remained unclear. However, the striking accumulation of anionic oligosaccharides in double knockout mice totally deficient in hexosaminidase activity but not in mice expressing Hex S (Sango, K., McDonald, M. P., Crawley, J. N., Mack, M. L., Tifft, C.J., Skop, E., Starr, C. M., Hoffmann, A., Sandhoff, K., Suzuki, K., and Proia, R. L., (1996) Nat. Genet. 14, 348-352) prompted us to reinvestigate the substrate specificity of Hex S. To identify physiological substrates of Hex S, anionic and neutral oligosaccharides excreted in the urine of the double knockout mice were isolated and analyzed. Using ESI-MS/MS and glycosidase digestion the anionic glycans were identified as products of incomplete dermatan sulfate degradation whereas the neutral storage oligosaccharides were found to be fragments of N-glycan degradation. In vitro, recombinant Hex S was highly active on water-soluble and amphiphilic glycoconjugates including artificial substrates, sulfated GAG fragments, and the sulfated glycosphingolipid SM2. Hydrolysis of membrane-bound SM2 by the recombinant Hex S was synergistically stimulated by the GM2 activator protein and the lysosomal anionic phospholipid bis(monoacylglycero)phosphate. << Less
J. Biol. Chem. 277:2562-2572(2002) [PubMed] [EuropePMC]
This publication is cited by 2 other entries.