1I3N
MOLECULAR BASIS FOR SEVERE EPIMERASE-DEFICIENCY GALACTOSEMIA: X-RAY STRUCTURE OF THE HUMAN V94M-SUBSTITUTED UDP-GALACTOSE 4-EPIMERASE
Functional Information from GO Data
Chain | GOid | namespace | contents |
A | 0003974 | molecular_function | UDP-N-acetylglucosamine 4-epimerase activity |
A | 0003978 | molecular_function | UDP-glucose 4-epimerase activity |
A | 0005829 | cellular_component | cytosol |
A | 0006012 | biological_process | galactose metabolic process |
A | 0016853 | molecular_function | isomerase activity |
A | 0019388 | biological_process | galactose catabolic process |
A | 0033499 | biological_process | galactose catabolic process via UDP-galactose |
A | 0042802 | molecular_function | identical protein binding |
A | 0042803 | molecular_function | protein homodimerization activity |
B | 0003974 | molecular_function | UDP-N-acetylglucosamine 4-epimerase activity |
B | 0003978 | molecular_function | UDP-glucose 4-epimerase activity |
B | 0005829 | cellular_component | cytosol |
B | 0006012 | biological_process | galactose metabolic process |
B | 0016853 | molecular_function | isomerase activity |
B | 0019388 | biological_process | galactose catabolic process |
B | 0033499 | biological_process | galactose catabolic process via UDP-galactose |
B | 0042802 | molecular_function | identical protein binding |
B | 0042803 | molecular_function | protein homodimerization activity |
Functional Information from PDB Data
site_id | AC1 |
Number of Residues | 4 |
Details | BINDING SITE FOR RESIDUE CL A 990 |
Chain | Residue |
A | GLY43 |
A | SER44 |
A | LEU45 |
A | HOH1949 |
site_id | AC2 |
Number of Residues | 4 |
Details | BINDING SITE FOR RESIDUE CL B 991 |
Chain | Residue |
A | LYS120 |
A | HOH1438 |
A | HOH1641 |
B | LEU102 |
site_id | AC3 |
Number of Residues | 1 |
Details | BINDING SITE FOR RESIDUE CL A 992 |
Chain | Residue |
A | ASP231 |
site_id | AC4 |
Number of Residues | 3 |
Details | BINDING SITE FOR RESIDUE CL B 993 |
Chain | Residue |
B | SER44 |
B | LEU45 |
B | ARG50 |
site_id | AC5 |
Number of Residues | 6 |
Details | BINDING SITE FOR RESIDUE MG A 994 |
Chain | Residue |
A | SER312 |
A | HOH1174 |
A | HOH1463 |
A | HOH1565 |
A | HOH1953 |
A | HOH1954 |
site_id | AC6 |
Number of Residues | 35 |
Details | BINDING SITE FOR RESIDUE NAD A 400 |
Chain | Residue |
A | GLY9 |
A | GLY12 |
A | TYR13 |
A | ILE14 |
A | ASP33 |
A | ASN34 |
A | HIS36 |
A | ASN37 |
A | ALA38 |
A | MET65 |
A | ASP66 |
A | ILE67 |
A | PHE88 |
A | ALA89 |
A | GLY90 |
A | LYS92 |
A | VAL107 |
A | SER130 |
A | SER131 |
A | SER132 |
A | TYR157 |
A | LYS161 |
A | TYR185 |
A | PRO188 |
A | HOH1101 |
A | HOH1111 |
A | HOH1113 |
A | HOH1152 |
A | HOH1153 |
A | HOH1347 |
A | HOH1359 |
A | HOH1388 |
A | HOH1788 |
A | HOH1789 |
A | HOH1934 |
site_id | AC7 |
Number of Residues | 22 |
Details | BINDING SITE FOR RESIDUE UD1 A 401 |
Chain | Residue |
A | ALA93 |
A | ASN187 |
A | ASN206 |
A | ASN207 |
A | LEU208 |
A | ASN224 |
A | VAL225 |
A | PHE226 |
A | GLY237 |
A | ARG239 |
A | TYR241 |
A | VAL277 |
A | ARG300 |
A | GLY302 |
A | ASP303 |
A | VAL304 |
A | HOH1194 |
A | HOH1483 |
A | HOH1504 |
A | HOH1510 |
A | HOH1819 |
A | HOH1926 |
site_id | AC8 |
Number of Residues | 32 |
Details | BINDING SITE FOR RESIDUE NAD B 900 |
Chain | Residue |
B | HOH1252 |
B | HOH1264 |
B | HOH1276 |
B | HOH1277 |
B | HOH1589 |
B | HOH1601 |
B | HOH1837 |
B | HOH1935 |
B | GLY9 |
B | GLY12 |
B | TYR13 |
B | ILE14 |
B | ASP33 |
B | ASN34 |
B | HIS36 |
B | ASN37 |
B | ALA38 |
B | MET65 |
B | ASP66 |
B | ILE67 |
B | PHE88 |
B | ALA89 |
B | GLY90 |
B | LYS92 |
B | SER130 |
B | SER131 |
B | TYR157 |
B | LYS161 |
B | TYR185 |
B | PRO188 |
B | UD1901 |
B | HOH1246 |
site_id | AC9 |
Number of Residues | 21 |
Details | BINDING SITE FOR RESIDUE UD1 B 901 |
Chain | Residue |
B | TYR157 |
B | ASN187 |
B | ASN206 |
B | ASN207 |
B | LEU208 |
B | ASN224 |
B | VAL225 |
B | PHE226 |
B | GLY237 |
B | ARG239 |
B | TYR241 |
B | VAL277 |
B | ARG300 |
B | ASP303 |
B | NAD900 |
B | HOH1695 |
B | HOH1698 |
B | HOH1716 |
B | HOH1906 |
B | HOH1940 |
B | HOH1985 |
Functional Information from SwissProt/UniProt
site_id | SWS_FT_FI1 |
Number of Residues | 2 |
Details | ACT_SITE: Proton acceptor => ECO:0000269|PubMed:10801319, ECO:0000269|PubMed:11279032, ECO:0000269|PubMed:11279193, ECO:0000303|PubMed:15175331 |
Chain | Residue | Details |
A | TYR157 | |
B | TYR157 |
site_id | SWS_FT_FI2 |
Number of Residues | 18 |
Details | BINDING: BINDING => ECO:0000269|PubMed:10801319, ECO:0000269|PubMed:11279032, ECO:0000269|PubMed:11279193 |
Chain | Residue | Details |
A | GLY12 | |
B | GLY12 | |
B | ASP33 | |
B | ASP66 | |
B | PHE88 | |
B | LYS92 | |
B | LYS161 | |
B | ASN206 | |
B | ASN224 | |
B | ARG239 | |
A | ASP33 | |
A | ASP66 | |
A | PHE88 | |
A | LYS92 | |
A | LYS161 | |
A | ASN206 | |
A | ASN224 | |
A | ARG239 |
site_id | SWS_FT_FI3 |
Number of Residues | 2 |
Details | BINDING: BINDING => ECO:0000269|PubMed:10801319, ECO:0000269|PubMed:11279032, ECO:0000269|PubMed:11279193, ECO:0000303|PubMed:15175331 |
Chain | Residue | Details |
A | SER132 | |
B | SER132 |
site_id | SWS_FT_FI4 |
Number of Residues | 2 |
Details | BINDING: BINDING => ECO:0000269|PubMed:10801319 |
Chain | Residue | Details |
A | TYR185 | |
B | TYR185 |
site_id | SWS_FT_FI5 |
Number of Residues | 2 |
Details | BINDING: BINDING => ECO:0000269|PubMed:11279032, ECO:0000269|PubMed:11279193 |
Chain | Residue | Details |
A | ARG300 | |
B | ARG300 |
Catalytic Information from CSA
site_id | CSA1 |
Number of Residues | 3 |
Details | Annotated By Reference To The Literature 1eq2 |
Chain | Residue | Details |
A | LYS161 | |
A | TYR157 | |
A | SER132 |
site_id | CSA2 |
Number of Residues | 3 |
Details | Annotated By Reference To The Literature 1eq2 |
Chain | Residue | Details |
B | LYS161 | |
B | TYR157 | |
B | SER132 |
site_id | CSA3 |
Number of Residues | 4 |
Details | Annotated By Reference To The Literature 1eq2 |
Chain | Residue | Details |
A | LYS161 | |
A | SER130 | |
A | TYR157 | |
A | ASN108 |
site_id | CSA4 |
Number of Residues | 4 |
Details | Annotated By Reference To The Literature 1eq2 |
Chain | Residue | Details |
B | LYS161 | |
B | SER130 | |
B | TYR157 | |
B | ASN108 |
site_id | CSA5 |
Number of Residues | 2 |
Details | Annotated By Reference To The Literature 1eq2 |
Chain | Residue | Details |
A | TYR141 | |
A | LYS161 |
site_id | CSA6 |
Number of Residues | 2 |
Details | Annotated By Reference To The Literature 1eq2 |
Chain | Residue | Details |
B | TYR141 | |
B | LYS161 |
site_id | CSA7 |
Number of Residues | 2 |
Details | Annotated By Reference To The Literature 1eq2 |
Chain | Residue | Details |
A | LYS161 | |
A | TYR157 |
site_id | CSA8 |
Number of Residues | 2 |
Details | Annotated By Reference To The Literature 1eq2 |
Chain | Residue | Details |
B | LYS161 | |
B | TYR157 |