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TitleCrystallographic structure of human beta-Hexosaminidase A: Interpretation of Tay-Sachs Mutations and Loss of GM2 Ganglioside Hydrolysis
Journal, issue, pagesJ. Mol. Biol., Vol. 359, Page 913-929, Year 2006
Publish dateMar 31, 2006 (structure data deposition date)
AuthorsLemieux, M.J. / Mark, B.L. / Cherney, M.M. / Withers, S.G. / Mahuran, D.J. / James, M.N.G.
External linksJ. Mol. Biol. / PubMed:16698036
MethodsX-ray diffraction
Resolution2.8 - 3.25 Å
Structure data

PDB-2gjx:
Crystallographic structure of human beta-Hexosaminidase A
Method: X-RAY DIFFRACTION / Resolution: 2.8 Å

PDB-2gk1:
X-ray crystal structure of NGT-bound HexA
Method: X-RAY DIFFRACTION / Resolution: 3.25 Å

Chemicals

ChemComp-NAG:
2-acetamido-2-deoxy-beta-D-glucopyranose

ChemComp-SO4:
SULFATE ION

ChemComp-HOH:
WATER

ChemComp-NGT:
3AR,5R,6S,7R,7AR-5-HYDROXYMETHYL-2-METHYL-5,6,7,7A-TETRAHYDRO-3AH-PYRANO[3,2-D]THIAZOLE-6,7-DIOL

Source
  • homo sapiens (human)
KeywordsHYDROLASE / beta-hexosaminidase A / glycosidase / Tay-Sachs disease / GM2 ganglisode / Tim barrel / beta-hexoasaminidase A / Sandhoff disease / NAG-thazoline / GM2 gangliodosis

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