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2NSX

Structure of acid-beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease

Entity
Entity IDChain IDDescriptionTypeChain lengthFormula weightNumber of moleculesDB Name (Accession)Biological sourceDescriptive keywords
1A, B, C, DGlucosylceramidasepolymer49755640.24UniProt (P04062)
Pfam (PF02055)
Pfam (PF17189)
In PDB
Homo sapiens (human)Beta-glucocerebrosidase, Acid beta-glucosidase, D-glucosyl-N-acylsphingosine glucohydrolase, Alglucerase, Imiglucerase
2A, B, C, D2-acetamido-2-deoxy-beta-D-glucopyranosenon-polymer221.25Chemie (NAG)
3D, A, B, CSULFATE IONnon-polymer96.118Chemie (SO4)
4A, CGLYCEROLnon-polymer92.12Chemie (GOL)
5D, B5-HYDROXYMETHYL-3,4-DIHYDROXYPIPERIDINEnon-polymer147.22Chemie (IFM)
6waterwater18.01006Chemie (HOH)
Sequence modifications
A, B, C, D: 1 - 497 (UniProt: P04062)
PDBExternal DatabaseDetails
His 495Arg 534conflict
Sequence viewer
Contents of the asymmetric unit
PolymersNumber of chains4
Total formula weight222560.7
Non-Polymers*Number of molecules27
Total formula weight3313.7
All*Total formula weight225874.4
*Water molecules are not included.

224004

PDB entries from 2024-08-21

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