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	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	0099128	cellular_component	mitochondrial [2Fe-2S] assembly complex
B	1990221	cellular_component	L-cysteine desulfurase complex
B	1990229	cellular_component	iron-sulfur cluster assembly 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	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	0099128	cellular_component	mitochondrial [2Fe-2S] assembly complex
F	1990221	cellular_component	L-cysteine desulfurase complex
F	1990229	cellular_component	iron-sulfur cluster assembly 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	ACT_SITE: Cysteine persulfide intermediate => ECO:0000250|UniProtKB:Q9D7P6

Chain	Residue	Details
D	CYS69
H	CYS69
F	ARG6
F	LYS44

---

site_id	SWS_FT_FI2
Number of Residues	2
Details	ACT_SITE: Cysteine persulfide intermediate => ECO:0000250|UniProtKB:O29689

Chain	Residue	Details
D	CYS138
H	CYS138

---

site_id	SWS_FT_FI3
Number of Residues	6
Details	BINDING: BINDING => ECO:0000269|PubMed:31101807, ECO:0007744|PDB:6NZU

Chain	Residue	Details
D	ASP71
D	CYS95
D	CYS138
H	ASP71
H	CYS95
H	CYS138
E	SER255
E	HIS257

---

site_id	SWS_FT_FI4
Number of Residues	2
Details	SITE: Mediates ISCU dimerization and de novo [2Fe-2S] cluster assembly => ECO:0000269|PubMed:34824239

Chain	Residue	Details
D	TYR35
H	TYR35

---

site_id	SWS_FT_FI5
Number of Residues	2
Details	MOD_RES: Cysteine persulfide => ECO:0000250|UniProtKB:Q9D7P6

Chain	Residue	Details
D	CYS69
H	CYS69

---

site_id	SWS_FT_FI6
Number of Residues	2
Details	MOD_RES: Cysteine persulfide => ECO:0000305|PubMed:24971490

Chain	Residue	Details
D	CYS138
H	CYS138

---

site_id	SWS_FT_FI7
Number of Residues	2
Details	MOD_RES: Cysteine persulfide => ECO:0000305|PubMed:18650437, ECO:0000305|PubMed:23593335

Chain	Residue	Details
A	CYS381
E	CYS381

---