5NN6
Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-hydroxyethyl-1-deoxynojirimycin
Functional Information from GO Data
| Chain | GOid | namespace | contents |
| A | 0000023 | biological_process | maltose metabolic process |
| A | 0002026 | biological_process | regulation of the force of heart contraction |
| A | 0002086 | biological_process | diaphragm contraction |
| A | 0003007 | biological_process | heart morphogenesis |
| A | 0003824 | molecular_function | catalytic activity |
| A | 0004553 | molecular_function | hydrolase activity, hydrolyzing O-glycosyl compounds |
| A | 0004558 | molecular_function | alpha-1,4-glucosidase activity |
| A | 0005764 | cellular_component | lysosome |
| A | 0005765 | cellular_component | lysosomal membrane |
| A | 0005886 | cellular_component | plasma membrane |
| A | 0005975 | biological_process | carbohydrate metabolic process |
| A | 0005977 | biological_process | glycogen metabolic process |
| A | 0005980 | biological_process | glycogen catabolic process |
| A | 0005985 | biological_process | sucrose metabolic process |
| A | 0006006 | biological_process | glucose metabolic process |
| A | 0006941 | biological_process | striated muscle contraction |
| A | 0007040 | biological_process | lysosome organization |
| A | 0007626 | biological_process | locomotory behavior |
| A | 0009888 | biological_process | tissue development |
| A | 0016020 | cellular_component | membrane |
| A | 0016798 | molecular_function | hydrolase activity, acting on glycosyl bonds |
| A | 0030246 | molecular_function | carbohydrate binding |
| A | 0032450 | molecular_function | maltose alpha-glucosidase activity |
| A | 0035577 | cellular_component | azurophil granule membrane |
| A | 0035904 | biological_process | aorta development |
| A | 0043181 | biological_process | vacuolar sequestering |
| A | 0043202 | cellular_component | lysosomal lumen |
| A | 0043231 | cellular_component | intracellular membrane-bounded organelle |
| A | 0046716 | biological_process | muscle cell cellular homeostasis |
| A | 0050884 | biological_process | neuromuscular process controlling posture |
| A | 0050885 | biological_process | neuromuscular process controlling balance |
| A | 0060048 | biological_process | cardiac muscle contraction |
| A | 0061723 | biological_process | glycophagy |
| A | 0070062 | cellular_component | extracellular exosome |
| A | 0070821 | cellular_component | tertiary granule membrane |
| A | 0090599 | molecular_function | alpha-glucosidase activity |
| A | 0101003 | cellular_component | ficolin-1-rich granule membrane |
| A | 0120282 | cellular_component | autolysosome lumen |
Functional Information from PROSITE/UniProt
| site_id | PS00025 |
| Number of Residues | 22 |
| Details | P_TREFOIL_1 P-type 'Trefoil' domain signature. RfdCaPdkaiTqeqCeargCCY |
| Chain | Residue | Details |
| A | ARG89-TYR110 |
| site_id | PS00129 |
| Number of Residues | 8 |
| Details | GLYCOSYL_HYDROL_F31_1 Glycosyl hydrolases family 31 active site. GMWiDMNE |
| Chain | Residue | Details |
| A | GLY514-GLU521 |
| site_id | PS00707 |
| Number of Residues | 31 |
| Details | GLYCOSYL_HYDROL_F31_2 Glycosyl hydrolases family 31 signature 2. GADVCGFlgnTseeLCvRWtqLGAFyPFmRN |
| Chain | Residue | Details |
| A | GLY643-ASN673 |
Functional Information from SwissProt/UniProt
| site_id | SWS_FT_FI1 |
| Number of Residues | 1 |
| Details | ACT_SITE: Nucleophile => ECO:0000255|PROSITE-ProRule:PRU10066, ECO:0000269|PubMed:1856189, ECO:0000305|PubMed:29061980 |
| Chain | Residue | Details |
| A | ASP518 |
| site_id | SWS_FT_FI2 |
| Number of Residues | 1 |
| Details | ACT_SITE: ACT_SITE => ECO:0000250 |
| Chain | Residue | Details |
| A | GLU521 |
| site_id | SWS_FT_FI3 |
| Number of Residues | 4 |
| Details | BINDING: BINDING => ECO:0000305|PubMed:29061980 |
| Chain | Residue | Details |
| A | ASP404 | |
| A | ARG600 | |
| A | ASP616 | |
| A | HIS674 |
| site_id | SWS_FT_FI4 |
| Number of Residues | 2 |
| Details | CARBOHYD: N-linked (GlcNAc...) asparagine => ECO:0000269|PubMed:19159218, ECO:0000269|PubMed:29061980, ECO:0000269|PubMed:8435067, ECO:0000269|Ref.19, ECO:0007744|PDB:5KZW, ECO:0007744|PDB:5NN3 |
| Chain | Residue | Details |
| A | ASN140 | |
| A | ASN882 |
| site_id | SWS_FT_FI5 |
| Number of Residues | 2 |
| Details | CARBOHYD: N-linked (GlcNAc...) asparagine => ECO:0000269|PubMed:29061980, ECO:0000269|PubMed:8435067, ECO:0000269|Ref.19, ECO:0007744|PDB:5KZW, ECO:0007744|PDB:5NN3 |
| Chain | Residue | Details |
| A | ASN233 | |
| A | ASN652 |
| site_id | SWS_FT_FI6 |
| Number of Residues | 1 |
| Details | CARBOHYD: N-linked (GlcNAc...) asparagine => ECO:0000269|PubMed:16335952, ECO:0000269|PubMed:29061980, ECO:0000269|PubMed:8435067, ECO:0000269|Ref.19, ECO:0007744|PDB:5KZW, ECO:0007744|PDB:5NN3 |
| Chain | Residue | Details |
| A | ASN390 |
| site_id | SWS_FT_FI7 |
| Number of Residues | 1 |
| Details | CARBOHYD: N-linked (GlcNAc...) asparagine => ECO:0000269|PubMed:12754519, ECO:0000269|PubMed:19159218, ECO:0000269|PubMed:29061980, ECO:0000269|PubMed:8435067, ECO:0000269|Ref.19, ECO:0007744|PDB:5KZW, ECO:0007744|PDB:5NN3 |
| Chain | Residue | Details |
| A | ASN470 |
| site_id | SWS_FT_FI8 |
| Number of Residues | 1 |
| Details | CARBOHYD: N-linked (GlcNAc...) asparagine => ECO:0000269|PubMed:19159218, ECO:0000269|PubMed:8435067 |
| Chain | Residue | Details |
| A | ASN925 |
