Acid-beta-glucosidase

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Human acid-β-glucosidase (PDB code 1ogs)

3D structures of Acid-β-glucosidase

Updated on 03-October-2017

2wkl, 3gxd, 3gxi, 3gxm, 2v3f, 2nt0, 2nt1, 2j25, 2f61, 1ogs, 3rik – hABG – human
3ke0, 3keh – hABG (mutant)
2wcg, 3gxf, 2vt0, 2v3e, 2v3d, 1y7v, 3ril – hABG + inhibitor
2nsx – hABG + pharmacological chaperone
2xwd, 2xwe – hABG + nojirimycin derivative
5fjs – ABG – Thermoanaerobacterium xylanolyticum

References

  1. Dvir H, Harel M, McCarthy AA, Toker L, Silman I, Futerman AH, Sussman JL. X-ray structure of human acid-beta-glucosidase, the defective enzyme in Gaucher disease. EMBO Rep. 2003 Jul;4(7):704-9. PMID:12792654 doi:10.1038/sj.embor.embor873
  2. Grabowski GA. Gaucher disease and other storage disorders. Hematology Am Soc Hematol Educ Program. 2012;2012:13-8. doi:, 10.1182/asheducation-2012.1.13. PMID:23233555 doi:http://dx.doi.org/10.1182/asheducation-2012.1.13

Additional Resources

Metabolic Disorders
Carbohydrate Metabolism
Treatment of Gaucher disease

Proteopedia Page Contributors and Editors (what is this?)

Michal Harel, Boris Brumshtein, Alexander Berchansky, Joel L. Sussman, Eran Hodis, David Canner

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