5KZX
Crystal structure of human GAA
Entity
| Entity ID | Chain ID | Description | Type | Chain length | Formula weight | Number of molecules | DB Name (Accession) | Biological source | Descriptive keywords |
| 1 | A | Lysosomal alpha-glucosidase | polymer | 874 | 97141.8 | 1 | UniProt (P10253) Pfam (PF00088) Pfam (PF13802) Pfam (PF01055) Pfam (PF21365) In PDB | Homo sapiens (Human) | Acid maltase,Aglucosidase alfa |
| 2 | B | 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-[alpha-D-fucopyranose-(1-6)]2-acetamido-2-deoxy-beta-D-glucopyranose | branched | 570.5 | 1 | In PDB GlyTouCan (G38216KS) | |||
| 3 | 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 | 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 | alpha-D-fucopyranose-(1-6)-2-acetamido-2-deoxy-beta-D-glucopyranose | branched | 367.3 | 1 | In PDB GlyTouCan (G18989MS) | |||
| 6 | A | 2-acetamido-2-deoxy-beta-D-glucopyranose | non-polymer | 221.2 | 1 | Chemie (NAG) | |||
| 7 | A | alpha-D-glucopyranose | non-polymer | 180.2 | 2 | Chemie (GLC) | |||
| 8 | A | beta-D-glucopyranose | non-polymer | 180.2 | 2 | Chemie (BGC) | |||
| 9 | A | 1,2-ETHANEDIOL | non-polymer | 62.1 | 3 | Chemie (EDO) | |||
| 10 | A | TRIETHYLENE GLYCOL | non-polymer | 150.2 | 1 | Chemie (PGE) | |||
| 11 | A | DI(HYDROXYETHYL)ETHER | non-polymer | 106.1 | 2 | Chemie (PEG) | |||
| 12 | A | SULFATE ION | non-polymer | 96.1 | 3 | Chemie (SO4) | |||
| 13 | water | water | 18.0 | 792 | Chemie (HOH) |
Sequence modifications
A: 79 - 952 (UniProt: P10253)
| PDB | External Database | Details |
|---|---|---|
| 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 | 97141.8 | |
| Branched | Number of molecules | 5 |
| Total formula weight | 2373.2 | |
| Non-Polymers* | Number of molecules | 14 |
| Total formula weight | 1778.6 | |
| All* | Total formula weight | 101293.7 |
