6NZU
Structure of the human frataxin-bound iron-sulfur cluster assembly complex
Functional Information from GO Data
| Chain | GOid | namespace | contents |
| A | 0030170 | molecular_function | pyridoxal phosphate binding |
| A | 0031071 | molecular_function | cysteine desulfurase activity |
| A | 0044571 | biological_process | [2Fe-2S] cluster assembly |
| B | 0005198 | molecular_function | structural molecule activity |
| B | 0005515 | molecular_function | protein binding |
| B | 0005634 | cellular_component | nucleus |
| B | 0005739 | cellular_component | mitochondrion |
| B | 0005759 | cellular_component | mitochondrial matrix |
| B | 0016226 | biological_process | iron-sulfur cluster assembly |
| B | 0016604 | cellular_component | nuclear body |
| B | 0042803 | molecular_function | protein homodimerization activity |
| B | 0044571 | biological_process | [2Fe-2S] cluster assembly |
| B | 0044572 | biological_process | [4Fe-4S] cluster assembly |
| B | 0060090 | molecular_function | molecular adaptor activity |
| B | 0099128 | cellular_component | mitochondrial [2Fe-2S] assembly complex |
| B | 1990221 | cellular_component | L-cysteine desulfurase complex |
| C | 0006633 | biological_process | fatty acid biosynthetic process |
| D | 0005506 | molecular_function | iron ion binding |
| D | 0016226 | biological_process | iron-sulfur cluster assembly |
| D | 0051536 | molecular_function | iron-sulfur cluster binding |
| E | 0030170 | molecular_function | pyridoxal phosphate binding |
| E | 0031071 | molecular_function | cysteine desulfurase activity |
| E | 0044571 | biological_process | [2Fe-2S] cluster assembly |
| F | 0005198 | molecular_function | structural molecule activity |
| F | 0005515 | molecular_function | protein binding |
| F | 0005634 | cellular_component | nucleus |
| F | 0005739 | cellular_component | mitochondrion |
| F | 0005759 | cellular_component | mitochondrial matrix |
| F | 0016226 | biological_process | iron-sulfur cluster assembly |
| F | 0016604 | cellular_component | nuclear body |
| F | 0042803 | molecular_function | protein homodimerization activity |
| F | 0044571 | biological_process | [2Fe-2S] cluster assembly |
| F | 0044572 | biological_process | [4Fe-4S] cluster assembly |
| F | 0060090 | molecular_function | molecular adaptor activity |
| F | 0099128 | cellular_component | mitochondrial [2Fe-2S] assembly complex |
| F | 1990221 | cellular_component | L-cysteine desulfurase complex |
| G | 0006633 | biological_process | fatty acid biosynthetic process |
| H | 0005506 | molecular_function | iron ion binding |
| H | 0016226 | biological_process | iron-sulfur cluster assembly |
| H | 0051536 | molecular_function | iron-sulfur cluster binding |
| I | 0004322 | molecular_function | ferroxidase activity |
| I | 0005739 | cellular_component | mitochondrion |
| I | 0006879 | biological_process | intracellular iron ion homeostasis |
| I | 0008199 | molecular_function | ferric iron binding |
| I | 0016226 | biological_process | iron-sulfur cluster assembly |
| I | 0018283 | biological_process | iron incorporation into metallo-sulfur cluster |
| J | 0004322 | molecular_function | ferroxidase activity |
| J | 0005739 | cellular_component | mitochondrion |
| J | 0006879 | biological_process | intracellular iron ion homeostasis |
| J | 0008199 | molecular_function | ferric iron binding |
| J | 0016226 | biological_process | iron-sulfur cluster assembly |
| J | 0018283 | biological_process | iron incorporation into metallo-sulfur cluster |
Functional Information from PDB Data
| site_id | AC1 |
| Number of Residues | 9 |
| Details | binding site for residue PLP A 501 |
| Chain | Residue |
| A | GLY126 |
| A | ALA127 |
| A | THR128 |
| A | ASP232 |
| A | GLN235 |
| A | SER255 |
| A | HIS257 |
| A | LYS258 |
| E | THR295 |
| site_id | AC2 |
| Number of Residues | 7 |
| Details | binding site for residue 8Q1 C 101 |
| Chain | Residue |
| B | ARG6 |
| B | ALA39 |
| B | PHE40 |
| B | ASN43 |
| B | LYS44 |
| B | ASP62 |
| C | SER37 |
| site_id | AC3 |
| Number of Residues | 3 |
| Details | binding site for residue ZN D 201 |
| Chain | Residue |
| D | ASP71 |
| D | CYS95 |
| D | CYS138 |
| site_id | AC4 |
| Number of Residues | 9 |
| Details | binding site for residue PLP E 501 |
| Chain | Residue |
| A | THR295 |
| E | GLY126 |
| E | ALA127 |
| E | THR128 |
| E | ASP232 |
| E | GLN235 |
| E | SER255 |
| E | HIS257 |
| E | LYS258 |
| site_id | AC5 |
| Number of Residues | 3 |
| Details | binding site for residue ZN H 201 |
| Chain | Residue |
| H | ASP71 |
| H | CYS95 |
| H | CYS138 |
| site_id | AC6 |
| Number of Residues | 13 |
| Details | binding site for Di-peptide 8Q1 G 101 and SER G 37 |
| Chain | Residue |
| E | PRO71 |
| F | ARG6 |
| F | ALA39 |
| F | PHE40 |
| F | ASN43 |
| F | LYS44 |
| F | VAL46 |
| F | ASP62 |
| G | ASP36 |
| G | LEU38 |
| G | ASP39 |
| G | THR40 |
| G | VAL41 |
Functional Information from PROSITE/UniProt
| site_id | PS00012 |
| Number of Residues | 16 |
| Details | PHOSPHOPANTETHEINE Phosphopantetheine attachment site. DLGADSLDTVELVMAL |
| Chain | Residue | Details |
| C | ASP32-LEU47 |
| site_id | PS00595 |
| Number of Residues | 20 |
| Details | AA_TRANSFER_CLASS_5 Aminotransferases class-V pyridoxal-phosphate attachment site. IDLMsiSGHKiygpk.GvGaI |
| Chain | Residue | Details |
| A | ILE249-ILE268 |
| site_id | PS01344 |
| Number of Residues | 15 |
| Details | FRATAXIN_1 Frataxin family signature. VINKQtPnkQIWlSS |
| Chain | Residue | Details |
| I | VAL144-SER158 |
Functional Information from SwissProt/UniProt
| site_id | SWS_FT_FI1 |
| Number of Residues | 2 |
| Details | Active site: {"description":"Cysteine persulfide intermediate","evidences":[{"source":"PubMed","id":"18650437","evidenceCode":"ECO:0000305"},{"source":"PubMed","id":"23593335","evidenceCode":"ECO:0000305"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI2 |
| Number of Residues | 2 |
| Details | Binding site: {"evidences":[{"source":"PubMed","id":"29097656","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"34824239","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"5WKP","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"5WLW","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6UXE","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6W1D","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6WI2","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6WIH","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"7RTK","evidenceCode":"ECO:0007744"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI3 |
| Number of Residues | 8 |
| Details | Binding site: {"evidences":[{"source":"PubMed","id":"29097656","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"31101807","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"34824239","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"5WKP","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"5WLW","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6NZU","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6UXE","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6W1D","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6WI2","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6WIH","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"7RTK","evidenceCode":"ECO:0007744"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI4 |
| Number of Residues | 2 |
| Details | Binding site: {"evidences":[{"source":"PubMed","id":"29097656","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"31101807","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"5WKP","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"5WLW","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6NZU","evidenceCode":"ECO:0007744"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI5 |
| Number of Residues | 8 |
| Details | Binding site: {"evidences":[{"source":"PubMed","id":"29097656","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"5WLW","evidenceCode":"ECO:0007744"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI6 |
| Number of Residues | 2 |
| Details | Modified residue: {"description":"N6-(pyridoxal phosphate)lysine","evidences":[{"source":"PubMed","id":"29097656","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"31101807","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"34824239","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"5WKP","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"5WLW","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6NZU","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6UXE","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6W1D","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6WI2","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6WIH","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"7RTK","evidenceCode":"ECO:0007744"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI7 |
| Number of Residues | 2 |
| Details | Modified residue: {"description":"Cysteine persulfide","evidences":[{"source":"PubMed","id":"18650437","evidenceCode":"ECO:0000305"},{"source":"PubMed","id":"23593335","evidenceCode":"ECO:0000305"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI8 |
| Number of Residues | 4 |
| Details | Binding site: {"evidences":[{"source":"PubMed","id":"31664822","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"6ODD","evidenceCode":"ECO:0007744"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI9 |
| Number of Residues | 2 |
| Details | Modified residue: {"description":"N6-succinyllysine","evidences":[{"source":"UniProtKB","id":"Q8K215","evidenceCode":"ECO:0000250"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI10 |
| Number of Residues | 2 |
| Details | Active site: {"description":"Cysteine persulfide intermediate","evidences":[{"source":"UniProtKB","id":"Q9D7P6","evidenceCode":"ECO:0000250"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI11 |
| Number of Residues | 2 |
| Details | Active site: {"description":"Cysteine persulfide intermediate","evidences":[{"source":"UniProtKB","id":"O29689","evidenceCode":"ECO:0000250"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI12 |
| Number of Residues | 6 |
| Details | Binding site: {"evidences":[{"source":"PubMed","id":"31101807","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"6NZU","evidenceCode":"ECO:0007744"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI13 |
| Number of Residues | 2 |
| Details | Site: {"description":"Mediates ISCU dimerization and de novo [2Fe-2S] cluster assembly","evidences":[{"source":"PubMed","id":"34824239","evidenceCode":"ECO:0000269"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI14 |
| Number of Residues | 2 |
| Details | Modified residue: {"description":"Cysteine persulfide","evidences":[{"source":"UniProtKB","id":"Q9D7P6","evidenceCode":"ECO:0000250"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI15 |
| Number of Residues | 2 |
| Details | Modified residue: {"description":"Cysteine persulfide","evidences":[{"source":"PubMed","id":"24971490","evidenceCode":"ECO:0000305"}]} |
| Chain | Residue | Details |






