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6NZU

Structure of the human frataxin-bound iron-sulfur cluster assembly complex

Functional Information from GO Data
ChainGOidnamespacecontents
A0030170molecular_functionpyridoxal phosphate binding
A0031071molecular_functioncysteine desulfurase activity
A0044571biological_process[2Fe-2S] cluster assembly
B0005198molecular_functionstructural molecule activity
B0005515molecular_functionprotein binding
B0005634cellular_componentnucleus
B0005739cellular_componentmitochondrion
B0005759cellular_componentmitochondrial matrix
B0016226biological_processiron-sulfur cluster assembly
B0016604cellular_componentnuclear body
B0042803molecular_functionprotein homodimerization activity
B0044571biological_process[2Fe-2S] cluster assembly
B0044572biological_process[4Fe-4S] cluster assembly
B0060090molecular_functionmolecular adaptor activity
B0099128cellular_componentmitochondrial [2Fe-2S] assembly complex
B1990221cellular_componentL-cysteine desulfurase complex
C0006633biological_processfatty acid biosynthetic process
D0005506molecular_functioniron ion binding
D0016226biological_processiron-sulfur cluster assembly
D0051536molecular_functioniron-sulfur cluster binding
E0030170molecular_functionpyridoxal phosphate binding
E0031071molecular_functioncysteine desulfurase activity
E0044571biological_process[2Fe-2S] cluster assembly
F0005198molecular_functionstructural molecule activity
F0005515molecular_functionprotein binding
F0005634cellular_componentnucleus
F0005739cellular_componentmitochondrion
F0005759cellular_componentmitochondrial matrix
F0016226biological_processiron-sulfur cluster assembly
F0016604cellular_componentnuclear body
F0042803molecular_functionprotein homodimerization activity
F0044571biological_process[2Fe-2S] cluster assembly
F0044572biological_process[4Fe-4S] cluster assembly
F0060090molecular_functionmolecular adaptor activity
F0099128cellular_componentmitochondrial [2Fe-2S] assembly complex
F1990221cellular_componentL-cysteine desulfurase complex
G0006633biological_processfatty acid biosynthetic process
H0005506molecular_functioniron ion binding
H0016226biological_processiron-sulfur cluster assembly
H0051536molecular_functioniron-sulfur cluster binding
I0004322molecular_functionferroxidase activity
I0005739cellular_componentmitochondrion
I0006879biological_processintracellular iron ion homeostasis
I0008199molecular_functionferric iron binding
I0016226biological_processiron-sulfur cluster assembly
I0018283biological_processiron incorporation into metallo-sulfur cluster
J0004322molecular_functionferroxidase activity
J0005739cellular_componentmitochondrion
J0006879biological_processintracellular iron ion homeostasis
J0008199molecular_functionferric iron binding
J0016226biological_processiron-sulfur cluster assembly
J0018283biological_processiron incorporation into metallo-sulfur cluster
Functional Information from PDB Data
site_idAC1
Number of Residues9
Detailsbinding site for residue PLP A 501
ChainResidue
AGLY126
AALA127
ATHR128
AASP232
AGLN235
ASER255
AHIS257
ALYS258
ETHR295

site_idAC2
Number of Residues7
Detailsbinding site for residue 8Q1 C 101
ChainResidue
BARG6
BALA39
BPHE40
BASN43
BLYS44
BASP62
CSER37

site_idAC3
Number of Residues3
Detailsbinding site for residue ZN D 201
ChainResidue
DASP71
DCYS95
DCYS138

site_idAC4
Number of Residues9
Detailsbinding site for residue PLP E 501
ChainResidue
ATHR295
EGLY126
EALA127
ETHR128
EASP232
EGLN235
ESER255
EHIS257
ELYS258

site_idAC5
Number of Residues3
Detailsbinding site for residue ZN H 201
ChainResidue
HASP71
HCYS95
HCYS138

site_idAC6
Number of Residues13
Detailsbinding site for Di-peptide 8Q1 G 101 and SER G 37
ChainResidue
EPRO71
FARG6
FALA39
FPHE40
FASN43
FLYS44
FVAL46
FASP62
GASP36
GLEU38
GASP39
GTHR40
GVAL41

Functional Information from PROSITE/UniProt
site_idPS00012
Number of Residues16
DetailsPHOSPHOPANTETHEINE Phosphopantetheine attachment site. DLGADSLDTVELVMAL
ChainResidueDetails
CASP32-LEU47

site_idPS00595
Number of Residues20
DetailsAA_TRANSFER_CLASS_5 Aminotransferases class-V pyridoxal-phosphate attachment site. IDLMsiSGHKiygpk.GvGaI
ChainResidueDetails
AILE249-ILE268

site_idPS01344
Number of Residues15
DetailsFRATAXIN_1 Frataxin family signature. VINKQtPnkQIWlSS
ChainResidueDetails
IVAL144-SER158

Functional Information from SwissProt/UniProt
site_idSWS_FT_FI1
Number of Residues2
DetailsActive site: {"description":"Cysteine persulfide intermediate","evidences":[{"source":"PubMed","id":"18650437","evidenceCode":"ECO:0000305"},{"source":"PubMed","id":"23593335","evidenceCode":"ECO:0000305"}]}
ChainResidueDetails

site_idSWS_FT_FI2
Number of Residues2
DetailsBinding 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"}]}
ChainResidueDetails

site_idSWS_FT_FI3
Number of Residues8
DetailsBinding 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"}]}
ChainResidueDetails

site_idSWS_FT_FI4
Number of Residues2
DetailsBinding 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"}]}
ChainResidueDetails

site_idSWS_FT_FI5
Number of Residues8
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"29097656","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"5WLW","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

site_idSWS_FT_FI6
Number of Residues2
DetailsModified 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"}]}
ChainResidueDetails

site_idSWS_FT_FI7
Number of Residues2
DetailsModified residue: {"description":"Cysteine persulfide","evidences":[{"source":"PubMed","id":"18650437","evidenceCode":"ECO:0000305"},{"source":"PubMed","id":"23593335","evidenceCode":"ECO:0000305"}]}
ChainResidueDetails

site_idSWS_FT_FI8
Number of Residues4
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"31664822","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"6ODD","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

site_idSWS_FT_FI9
Number of Residues2
DetailsModified residue: {"description":"N6-succinyllysine","evidences":[{"source":"UniProtKB","id":"Q8K215","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails

site_idSWS_FT_FI10
Number of Residues2
DetailsActive site: {"description":"Cysteine persulfide intermediate","evidences":[{"source":"UniProtKB","id":"Q9D7P6","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails

site_idSWS_FT_FI11
Number of Residues2
DetailsActive site: {"description":"Cysteine persulfide intermediate","evidences":[{"source":"UniProtKB","id":"O29689","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails

site_idSWS_FT_FI12
Number of Residues6
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"31101807","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"6NZU","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

site_idSWS_FT_FI13
Number of Residues2
DetailsSite: {"description":"Mediates ISCU dimerization and de novo [2Fe-2S] cluster assembly","evidences":[{"source":"PubMed","id":"34824239","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails

site_idSWS_FT_FI14
Number of Residues2
DetailsModified residue: {"description":"Cysteine persulfide","evidences":[{"source":"UniProtKB","id":"Q9D7P6","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails

site_idSWS_FT_FI15
Number of Residues2
DetailsModified residue: {"description":"Cysteine persulfide","evidences":[{"source":"PubMed","id":"24971490","evidenceCode":"ECO:0000305"}]}
ChainResidueDetails

247536

PDB entries from 2026-01-14

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