2NSX
Structure of acid-beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease
Entity
| Entity ID | Chain ID | Description | Type | Chain length | Formula weight | Number of molecules | DB Name (Accession) | Biological source | Descriptive keywords |
| 1 | A, B, C, D (A, B, C, D) | Glucosylceramidase | polymer | 497 | 55640.2 | 4 | UniProt (P04062) Pfam (PF02055) Pfam (PF17189) | Homo sapiens (human) | Beta-glucocerebrosidase, Acid beta-glucosidase, D-glucosyl-N-acylsphingosine glucohydrolase, Alglucerase, Imiglucerase |
| 2 | E, L, M, U, Z (A, B, C, D) | 2-acetamido-2-deoxy-beta-D-glucopyranose | non-polymer | 221.2 | 5 | Chemie (NAG) | |||
| 3 | AA, BA, CA, DA, F... (D, A, B, C) | SULFATE ION | non-polymer | 96.1 | 18 | Chemie (SO4) | |||
| 4 | K, Y (A, C) | GLYCEROL | non-polymer | 92.1 | 2 | Chemie (GOL) | |||
| 5 | EA, T (D, B) | 5-HYDROXYMETHYL-3,4-DIHYDROXYPIPERIDINE | non-polymer | 147.2 | 2 | Chemie (IFM) | |||
| 6 | FA, GA, HA, IA (A, B, C, D) | water | water | 18.0 | 1006 | Chemie (HOH) |
Sequence modifications
A, B, C, D: 1 - 497 (UniProt: P04062)
| PDB | External Database | Details |
|---|---|---|
| His 495 | Arg 534 | conflict |
Sequence viewer
Contents of the asymmetric unit
| Polymers | Number of chains | 4 |
| Total formula weight | 222560.7 | |
| Non-Polymers* | Number of molecules | 27 |
| Total formula weight | 3313.7 | |
| All* | Total formula weight | 225874.4 |
