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) PubChem (753) | |||
| 5 | EA, T (D, B) | 5-HYDROXYMETHYL-3,4-DIHYDROXYPIPERIDINE | non-polymer | 147.2 | 2 | Chemie (IFM) PubChem (25178019) PubChem (44578588) PubChem (447607) PubChem (57181891) PubChem (57590942) PubChem (4452693) PubChem (89062053) PubChem (10240946) PubChem (10510991) PubChem (10820691) PubChem (10844524) PubChem (15235482) PubChem (130784980) PubChem (130905021) PubChem (174012304) | |||
| 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 |
