5NN6
Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-hydroxyethyl-1-deoxynojirimycin
Entity
| Entity ID | Chain ID | Description | Type | Chain length | Formula weight | Number of molecules | DB Name (Accession) | Biological source | Descriptive keywords |
| 1 | A (A) | Lysosomal alpha-glucosidase | polymer | 872 | 96978.6 | 1 | UniProt (P10253) Pfam (PF00088) Pfam (PF13802) Pfam (PF01055) Pfam (PF21365) | Homo sapiens (Human) | Acid maltase,Aglucosidase alfa |
| 2 | B (B) | 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-[alpha-L-fucopyranose-(1-6)]2-acetamido-2-deoxy-beta-D-glucopyranose | branched | 570.5 | 1 | In PDB GlyTouCan (G21290RB) | |||
| 3 | C, D (C, D) | 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose | branched | 424.4 | 2 | In PDB GlyTouCan (G42666HT) | |||
| 4 | E (E) | beta-D-mannopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose | branched | 586.5 | 1 | In PDB GlyTouCan (G15407YE) | |||
| 5 | F (F) | alpha-L-fucopyranose-(1-6)-2-acetamido-2-deoxy-beta-D-glucopyranose | branched | 367.3 | 1 | In PDB GlyTouCan (G86851RC) | |||
| 6 | G (A) | (2R,3R,4R,5S)-1-(2-hydroxyethyl)-2-(hydroxymethyl)piperidine-3,4,5-triol | non-polymer | 207.2 | 1 | Chemie (MIG) PubChem (51577) PubChem (441314) PubChem (6710690) PubChem (9794283) PubChem (12766463) PubChem (23622608) PubChem (40467206) PubChem (40467208) PubChem (40477191) PubChem (42052023) PubChem (45039933) PubChem (49759862) PubChem (51397099) PubChem (51400355) PubChem (124300736) PubChem (57369304) PubChem (57560749) PubChem (71225356) PubChem (69541599) PubChem (69821021) PubChem (71749029) PubChem (89813532) PubChem (93532308) PubChem (124397038) PubChem (124427653) PubChem (124582590) PubChem (134693994) PubChem (134853664) PubChem (145752189) PubChem (158646524) PubChem (168440872) PubChem (171042507) PubChem (173771382) | |||
| 7 | H, I, J, K, L (A) | SULFATE ION | non-polymer | 96.1 | 5 | Chemie (SO4) | |||
| 8 | M, N, O (A) | CHLORIDE ION | non-polymer | 35.5 | 3 | Chemie (CL) PubChem (312) | |||
| 9 | AA, BA, P, Q, R... (A) | 1,2-ETHANEDIOL | non-polymer | 62.1 | 13 | Chemie (EDO) | |||
| 10 | CA, DA, EA, FA (A) | TRIETHYLENE GLYCOL | non-polymer | 150.2 | 4 | Chemie (PGE) | |||
| 11 | GA, HA, IA (A) | GLYCEROL | non-polymer | 92.1 | 3 | Chemie (GOL) PubChem (753) | |||
| 12 | JA (A) | water | water | 18.0 | 633 | Chemie (HOH) |
Sequence modifications
A: 81 - 952 (UniProt: P10253)
| PDB | External Database | Details |
|---|---|---|
| Arg 199 | His 199 | conflict |
| His 223 | Arg 223 | conflict |
| Ile 780 | Val 780 | conflict |
Sequence viewer
Contents of the asymmetric unit
| Polymers | Number of chains | 1 |
| Total formula weight | 96978.6 | |
| Branched | Number of molecules | 5 |
| Total formula weight | 2373.2 | |
| Non-Polymers* | Number of molecules | 29 |
| Total formula weight | 2477.8 | |
| All* | Total formula weight | 101829.6 |






