5KZ5

Architecture of the Human Mitochondrial Iron-Sulfur Cluster Assembly Machinery: the Complex Formed by the Iron Donor, the Sulfur Donor, and the Scaffold

External Resource: Annotation

Domain Annotation: SCOP2 Classification
Domain Annotation: ECOD Classification
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

Chains	Type	Family Name	Domain Identifier	Family Identifier	Provenance Source (Version)
A [auth 1]	SCOP2B Superfamily	PLP-dependent transferases	8074674	3000954	SCOP2B (2022-06-29)
B [auth 2]	SCOP2B Superfamily	PLP-dependent transferases	8074674	3000954	SCOP2B (2022-06-29)
C [auth 3]	SCOP2B Superfamily	PLP-dependent transferases	8074674	3000954	SCOP2B (2022-06-29)
D [auth 4]	SCOP2B Superfamily	PLP-dependent transferases	8074674	3000954	SCOP2B (2022-06-29)
E [auth M]	SCOP2B Superfamily	PLP-dependent transferases	8074674	3000954	SCOP2B (2022-06-29)
F [auth N]	SCOP2B Superfamily	PLP-dependent transferases	8074674	3000954	SCOP2B (2022-06-29)
G [auth O]	SCOP2B Superfamily	PLP-dependent transferases	8074674	3000954	SCOP2B (2022-06-29)
H [auth P]	SCOP2B Superfamily	PLP-dependent transferases	8074674	3000954	SCOP2B (2022-06-29)
I [auth Q]	SCOP2B Superfamily	PLP-dependent transferases	8074674	3000954	SCOP2B (2022-06-29)
J [auth R]	SCOP2B Superfamily	PLP-dependent transferases	8074674	3000954	SCOP2B (2022-06-29)
K [auth S]	SCOP2B Superfamily	PLP-dependent transferases	8074674	3000954	SCOP2B (2022-06-29)
L [auth T]	SCOP2B Superfamily	PLP-dependent transferases	8074674	3000954	SCOP2B (2022-06-29)
AA [auth H]	SCOP2B Superfamily	Frataxin/Nqo15-like	8035182	3001840	SCOP2B (2022-06-29)
CA [auth I]	SCOP2B Superfamily	Frataxin/Nqo15-like	8035182	3001840	SCOP2B (2022-06-29)
EA [auth J]	SCOP2B Superfamily	Frataxin/Nqo15-like	8035182	3001840	SCOP2B (2022-06-29)
GA [auth K]	SCOP2B Superfamily	Frataxin/Nqo15-like	8035182	3001840	SCOP2B (2022-06-29)
IA [auth L]	SCOP2B Superfamily	Frataxin/Nqo15-like	8035182	3001840	SCOP2B (2022-06-29)
M [auth A]	SCOP2B Superfamily	Frataxin/Nqo15-like	8035182	3001840	SCOP2B (2022-06-29)
O [auth B]	SCOP2B Superfamily	Frataxin/Nqo15-like	8035182	3001840	SCOP2B (2022-06-29)
Q [auth C]	SCOP2B Superfamily	Frataxin/Nqo15-like	8035182	3001840	SCOP2B (2022-06-29)
S [auth D]	SCOP2B Superfamily	Frataxin/Nqo15-like	8035182	3001840	SCOP2B (2022-06-29)
U [auth E]	SCOP2B Superfamily	Frataxin/Nqo15-like	8035182	3001840	SCOP2B (2022-06-29)
W [auth F]	SCOP2B Superfamily	Frataxin/Nqo15-like	8035182	3001840	SCOP2B (2022-06-29)
Y [auth G]	SCOP2B Superfamily	Frataxin/Nqo15-like	8035182	3001840	SCOP2B (2022-06-29)

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
A [auth 1]	PF00266	e5kz512	A: a+b two layers	X: C-terminal domain in some PLP-dependent transferases (From Topology)	H: C-terminal domain in some PLP-dependent transferases (From Topology)	T: C-terminal domain in some PLP-dependent transferases	F: PF00266	ECOD (1.6)
A [auth 1]	PF00266	e5kz511	A: a/b three-layered sandwiches	X: PLP-dependent transferases (From Topology)	H: PLP-dependent transferases (From Topology)	T: PLP-dependent transferases	F: PF00266	ECOD (1.6)
B [auth 2]	PF00266	e5kz522	A: a+b two layers	X: C-terminal domain in some PLP-dependent transferases (From Topology)	H: C-terminal domain in some PLP-dependent transferases (From Topology)	T: C-terminal domain in some PLP-dependent transferases	F: PF00266	ECOD (1.6)
B [auth 2]	PF00266	e5kz521	A: a/b three-layered sandwiches	X: PLP-dependent transferases (From Topology)	H: PLP-dependent transferases (From Topology)	T: PLP-dependent transferases	F: PF00266	ECOD (1.6)
C [auth 3]	PF00266	e5kz532	A: a+b two layers	X: C-terminal domain in some PLP-dependent transferases (From Topology)	H: C-terminal domain in some PLP-dependent transferases (From Topology)	T: C-terminal domain in some PLP-dependent transferases	F: PF00266	ECOD (1.6)
C [auth 3]	PF00266	e5kz531	A: a/b three-layered sandwiches	X: PLP-dependent transferases (From Topology)	H: PLP-dependent transferases (From Topology)	T: PLP-dependent transferases	F: PF00266	ECOD (1.6)
D [auth 4]	PF00266	e5kz541	A: a+b two layers	X: C-terminal domain in some PLP-dependent transferases (From Topology)	H: C-terminal domain in some PLP-dependent transferases (From Topology)	T: C-terminal domain in some PLP-dependent transferases	F: PF00266	ECOD (1.6)
D [auth 4]	PF00266	e5kz542	A: a/b three-layered sandwiches	X: PLP-dependent transferases (From Topology)	H: PLP-dependent transferases (From Topology)	T: PLP-dependent transferases	F: PF00266	ECOD (1.6)
E [auth M]	PF00266	e5kz5M2	A: a+b two layers	X: C-terminal domain in some PLP-dependent transferases (From Topology)	H: C-terminal domain in some PLP-dependent transferases (From Topology)	T: C-terminal domain in some PLP-dependent transferases	F: PF00266	ECOD (1.6)
E [auth M]	PF00266	e5kz5M1	A: a/b three-layered sandwiches	X: PLP-dependent transferases (From Topology)	H: PLP-dependent transferases (From Topology)	T: PLP-dependent transferases	F: PF00266	ECOD (1.6)
F [auth N]	PF00266	e5kz5N2	A: a+b two layers	X: C-terminal domain in some PLP-dependent transferases (From Topology)	H: C-terminal domain in some PLP-dependent transferases (From Topology)	T: C-terminal domain in some PLP-dependent transferases	F: PF00266	ECOD (1.6)
F [auth N]	PF00266	e5kz5N1	A: a/b three-layered sandwiches	X: PLP-dependent transferases (From Topology)	H: PLP-dependent transferases (From Topology)	T: PLP-dependent transferases	F: PF00266	ECOD (1.6)
G [auth O]	PF00266	e5kz5O2	A: a+b two layers	X: C-terminal domain in some PLP-dependent transferases (From Topology)	H: C-terminal domain in some PLP-dependent transferases (From Topology)	T: C-terminal domain in some PLP-dependent transferases	F: PF00266	ECOD (1.6)
G [auth O]	PF00266	e5kz5O1	A: a/b three-layered sandwiches	X: PLP-dependent transferases (From Topology)	H: PLP-dependent transferases (From Topology)	T: PLP-dependent transferases	F: PF00266	ECOD (1.6)
H [auth P]	PF00266	e5kz5P2	A: a+b two layers	X: C-terminal domain in some PLP-dependent transferases (From Topology)	H: C-terminal domain in some PLP-dependent transferases (From Topology)	T: C-terminal domain in some PLP-dependent transferases	F: PF00266	ECOD (1.6)
H [auth P]	PF00266	e5kz5P1	A: a/b three-layered sandwiches	X: PLP-dependent transferases (From Topology)	H: PLP-dependent transferases (From Topology)	T: PLP-dependent transferases	F: PF00266	ECOD (1.6)
I [auth Q]	PF00266	e5kz5Q2	A: a+b two layers	X: C-terminal domain in some PLP-dependent transferases (From Topology)	H: C-terminal domain in some PLP-dependent transferases (From Topology)	T: C-terminal domain in some PLP-dependent transferases	F: PF00266	ECOD (1.6)
I [auth Q]	PF00266	e5kz5Q1	A: a/b three-layered sandwiches	X: PLP-dependent transferases (From Topology)	H: PLP-dependent transferases (From Topology)	T: PLP-dependent transferases	F: PF00266	ECOD (1.6)
J [auth R]	PF00266	e5kz5R1	A: a+b two layers	X: C-terminal domain in some PLP-dependent transferases (From Topology)	H: C-terminal domain in some PLP-dependent transferases (From Topology)	T: C-terminal domain in some PLP-dependent transferases	F: PF00266	ECOD (1.6)
J [auth R]	PF00266	e5kz5R2	A: a/b three-layered sandwiches	X: PLP-dependent transferases (From Topology)	H: PLP-dependent transferases (From Topology)	T: PLP-dependent transferases	F: PF00266	ECOD (1.6)
K [auth S]	PF00266	e5kz5S2	A: a+b two layers	X: C-terminal domain in some PLP-dependent transferases (From Topology)	H: C-terminal domain in some PLP-dependent transferases (From Topology)	T: C-terminal domain in some PLP-dependent transferases	F: PF00266	ECOD (1.6)
K [auth S]	PF00266	e5kz5S1	A: a/b three-layered sandwiches	X: PLP-dependent transferases (From Topology)	H: PLP-dependent transferases (From Topology)	T: PLP-dependent transferases	F: PF00266	ECOD (1.6)
L [auth T]	PF00266	e5kz5T1	A: a+b two layers	X: C-terminal domain in some PLP-dependent transferases (From Topology)	H: C-terminal domain in some PLP-dependent transferases (From Topology)	T: C-terminal domain in some PLP-dependent transferases	F: PF00266	ECOD (1.6)
L [auth T]	PF00266	e5kz5T2	A: a/b three-layered sandwiches	X: PLP-dependent transferases (From Topology)	H: PLP-dependent transferases (From Topology)	T: PLP-dependent transferases	F: PF00266	ECOD (1.6)
AA [auth H]	PF01491	e5kz5H1	A: a+b two layers	X: Type III secretory system chaperone-like	H: Frataxin-like (From Topology)	T: Frataxin-like	F: PF01491	ECOD (1.6)
CA [auth I]	PF01491	e5kz5I1	A: a+b two layers	X: Type III secretory system chaperone-like	H: Frataxin-like (From Topology)	T: Frataxin-like	F: PF01491	ECOD (1.6)
EA [auth J]	PF01491	e5kz5J1	A: a+b two layers	X: Type III secretory system chaperone-like	H: Frataxin-like (From Topology)	T: Frataxin-like	F: PF01491	ECOD (1.6)
GA [auth K]	PF01491	e5kz5K1	A: a+b two layers	X: Type III secretory system chaperone-like	H: Frataxin-like (From Topology)	T: Frataxin-like	F: PF01491	ECOD (1.6)
IA [auth L]	PF01491	e5kz5L1	A: a+b two layers	X: Type III secretory system chaperone-like	H: Frataxin-like (From Topology)	T: Frataxin-like	F: PF01491	ECOD (1.6)
O [auth B]	PF01491	e5kz5B1	A: a+b two layers	X: Type III secretory system chaperone-like	H: Frataxin-like (From Topology)	T: Frataxin-like	F: PF01491	ECOD (1.6)
Q [auth C]	PF01491	e5kz5C1	A: a+b two layers	X: Type III secretory system chaperone-like	H: Frataxin-like (From Topology)	T: Frataxin-like	F: PF01491	ECOD (1.6)
S [auth D]	PF01491	e5kz5D1	A: a+b two layers	X: Type III secretory system chaperone-like	H: Frataxin-like (From Topology)	T: Frataxin-like	F: PF01491	ECOD (1.6)
U [auth E]	PF01491	e5kz5E1	A: a+b two layers	X: Type III secretory system chaperone-like	H: Frataxin-like (From Topology)	T: Frataxin-like	F: PF01491	ECOD (1.6)
W [auth F]	PF01491	e5kz5F1	A: a+b two layers	X: Type III secretory system chaperone-like	H: Frataxin-like (From Topology)	T: Frataxin-like	F: PF01491	ECOD (1.6)
Y [auth G]	PF01491	e5kz5G1	A: a+b two layers	X: Type III secretory system chaperone-like	H: Frataxin-like (From Topology)	T: Frataxin-like	F: PF01491	ECOD (1.6)
BA [auth h]	PF01592	e5kz5h1	A: a+b two layers	X: FAD-linked reductases, C-terminal domain-like	H: SufE/NifU (From Topology)	T: SufE/NifU	F: PF01592	ECOD (1.6)
DA [auth i]	PF01592	e5kz5i1	A: a+b two layers	X: FAD-linked reductases, C-terminal domain-like	H: SufE/NifU (From Topology)	T: SufE/NifU	F: PF01592	ECOD (1.6)
FA [auth j]	PF01592	e5kz5j1	A: a+b two layers	X: FAD-linked reductases, C-terminal domain-like	H: SufE/NifU (From Topology)	T: SufE/NifU	F: PF01592	ECOD (1.6)
HA [auth k]	PF01592	e5kz5k1	A: a+b two layers	X: FAD-linked reductases, C-terminal domain-like	H: SufE/NifU (From Topology)	T: SufE/NifU	F: PF01592	ECOD (1.6)
JA [auth l]	PF01592	e5kz5l1	A: a+b two layers	X: FAD-linked reductases, C-terminal domain-like	H: SufE/NifU (From Topology)	T: SufE/NifU	F: PF01592	ECOD (1.6)
N [auth a]	PF01592	e5kz5a1	A: a+b two layers	X: FAD-linked reductases, C-terminal domain-like	H: SufE/NifU (From Topology)	T: SufE/NifU	F: PF01592	ECOD (1.6)
P [auth b]	PF01592	e5kz5b1	A: a+b two layers	X: FAD-linked reductases, C-terminal domain-like	H: SufE/NifU (From Topology)	T: SufE/NifU	F: PF01592	ECOD (1.6)
R [auth c]	PF01592	e5kz5c1	A: a+b two layers	X: FAD-linked reductases, C-terminal domain-like	H: SufE/NifU (From Topology)	T: SufE/NifU	F: PF01592	ECOD (1.6)
T [auth d]	PF01592	e5kz5d1	A: a+b two layers	X: FAD-linked reductases, C-terminal domain-like	H: SufE/NifU (From Topology)	T: SufE/NifU	F: PF01592	ECOD (1.6)
V [auth e]	PF01592	e5kz5e1	A: a+b two layers	X: FAD-linked reductases, C-terminal domain-like	H: SufE/NifU (From Topology)	T: SufE/NifU	F: PF01592	ECOD (1.6)
X [auth f]	PF01592	e5kz5f1	A: a+b two layers	X: FAD-linked reductases, C-terminal domain-like	H: SufE/NifU (From Topology)	T: SufE/NifU	F: PF01592	ECOD (1.6)
Z [auth g]	PF01592	e5kz5g1	A: a+b two layers	X: FAD-linked reductases, C-terminal domain-like	H: SufE/NifU (From Topology)	T: SufE/NifU	F: PF01592	ECOD (1.6)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], F [auth N], G [auth O], H [auth P], I [auth Q], J [auth R], K [auth S], L [auth T]	PF00266	Aminotransferase class-V (Aminotran_5)	Aminotransferase class-V	This domain is found in amino transferases, and other enzymes including cysteine desulphurase EC:4.4.1.-.	Domain
AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], M [auth A], O [auth B], Q [auth C], S [auth D], U [auth E], W [auth F], Y [auth G]	PF01491	Frataxin-like domain (Frataxin_Cyay)	Frataxin-like domain	This family contains proteins that have a domain related to the globular C-terminus of Frataxin the protein that is mutated in Friedreich's ataxia. This domain is found in a family of bacterial proteins. The function of this domain is current ...	This family contains proteins that have a domain related to the globular C-terminus of Frataxin the protein that is mutated in Friedreich's ataxia. This domain is found in a family of bacterial proteins. The function of this domain is currently unknown. It has been suggested that this family is involved in iron transport.	Domain
BA [auth h], DA [auth i], FA [auth j], HA [auth k], JA [auth l], BA [auth h], DA [auth i], FA [auth j], HA [auth k], JA [auth l], N [auth a], P [auth b], R [auth c], T [auth d], V [auth e], X [auth f], Z [auth g]	PF01592	NifU-like N terminal domain (NifU_N)	NifU-like N terminal domain	-	Family

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], F [auth N], G [auth O], H [auth P], I [auth Q], J [auth R], K [auth S], L [auth T]	Cysteine desulfurase, mitochondrial	cysteine desulfurase activity sulfurtransferase activity transferase activity, transferring sulphur-containing groups transferase activity catalytic activity iron-sulfur cluster binding binding metal cluster binding small molecule binding vitamin binding vitamin B6 binding pyridoxal phosphate binding ion binding organic cyclic compound binding cysteine desulfurase activity sulfurtransferase activity transferase activity, transferring sulphur-containing groups transferase activity catalytic activity iron-sulfur cluster binding binding metal cluster binding small molecule binding vitamin binding vitamin B6 binding pyridoxal phosphate binding ion binding organic cyclic compound binding anion binding heterocyclic compound binding identical protein binding protein binding protein homodimerization activity protein dimerization activity cation binding metal ion binding	cellular component biogenesis cellular component organization metallo-sulfur cluster assembly cellular component assembly iron-sulfur cluster assembly cellular process cellular component organization or biogenesis organonitrogen compound metabolic process cellular metabolic process primary metabolic process prosthetic group metabolic process organic cyclic compound metabolic process organic substance metabolic process organophosphate metabolic process cellular component biogenesis cellular component organization metallo-sulfur cluster assembly cellular component assembly iron-sulfur cluster assembly cellular process cellular component organization or biogenesis organonitrogen compound metabolic process cellular metabolic process primary metabolic process prosthetic group metabolic process organic cyclic compound metabolic process organic substance metabolic process organophosphate metabolic process molybdopterin cofactor metabolic process phosphate-containing compound metabolic process metabolic process protein metabolic process macromolecule metabolic process phosphorus metabolic process [4Fe-4S] cluster assembly iron incorporation into metallo-sulfur cluster metal incorporation into metallo-sulfur cluster macromolecule modification protein modification process Mo-molybdopterin cofactor biosynthetic process Mo-molybdopterin cofactor metabolic process organonitrogen compound biosynthetic process molybdopterin cofactor biosynthetic process biosynthetic process organophosphate biosynthetic process organic cyclic compound biosynthetic process organic substance biosynthetic process [2Fe-2S] cluster assembly	membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle protein-containing complex cytosol iron-sulfur cluster assembly complex mitochondrial protein-containing complex mitochondrial iron-sulfur cluster assembly complex centrosome membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle protein-containing complex cytosol iron-sulfur cluster assembly complex mitochondrial protein-containing complex mitochondrial iron-sulfur cluster assembly complex centrosome microtubule organizing center cytoskeleton intracellular non-membrane-bounded organelle microtubule cytoskeleton non-membrane-bounded organelle nucleus membrane-enclosed lumen intracellular organelle lumen nucleoplasm organelle lumen nuclear lumen mitochondrial matrix
AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], M [auth A], O [auth B], Q [auth C], S [auth D], U [auth E], W [auth F], Y [auth G]	Frataxin, mitochondrial	cation binding transition metal ion binding iron ion binding ion binding binding ferrous iron binding metal ion binding small molecule binding ferric iron binding metallochaperone activity iron chaperone activity molecular carrier activity oxidoreductase activity, acting on metal ions oxidoreductase activity cation binding transition metal ion binding iron ion binding ion binding binding ferrous iron binding metal ion binding small molecule binding ferric iron binding metallochaperone activity iron chaperone activity molecular carrier activity oxidoreductase activity, acting on metal ions oxidoreductase activity ferroxidase activity oxidoreductase activity, acting on metal ions, oxygen as acceptor catalytic activity iron-sulfur cluster binding 2 iron, 2 sulfur cluster binding metal cluster binding	intracellular iron ion homeostasis inorganic ion homeostasis cellular homeostasis chemical homeostasis homeostatic process intracellular monoatomic cation homeostasis monoatomic ion homeostasis intracellular chemical homeostasis monoatomic cation homeostasis intracellular monoatomic ion homeostasis regulation of lyase activity regulation of molecular function positive regulation of molecular function regulation of catalytic activity intracellular iron ion homeostasis inorganic ion homeostasis cellular homeostasis chemical homeostasis homeostatic process intracellular monoatomic cation homeostasis monoatomic ion homeostasis intracellular chemical homeostasis monoatomic cation homeostasis intracellular monoatomic ion homeostasis regulation of lyase activity regulation of molecular function positive regulation of molecular function regulation of catalytic activity biological regulation positive regulation of catalytic activity positive regulation of aconitate hydratase activity regulation of aconitate hydratase activity positive regulation of lyase activity regulation of biological process regulation of growth regulation of cellular process regulation of cellular component organization positive regulation of biological process growth positive regulation of cell growth regulation of cell growth cell growth cellular component organization positive regulation of growth cellular process cellular component organization or biogenesis positive regulation of cellular process organonitrogen compound metabolic process cellular biosynthetic process cellular metabolic process primary metabolic process gene expression macromolecule biosynthetic process protein maturation organic substance metabolic process protein autoprocessing biosynthetic process proteolysis organic substance biosynthetic process protein processing metabolic process protein metabolic process macromolecule metabolic process localization transport iron ion transport transition metal ion transport monoatomic ion transport monoatomic cation transport establishment of localization metal ion transport developmental process multicellular organismal process developmental growth organ growth system process nervous system process sensory perception neuromuscular process proprioception neuromuscular process controlling posture cellular component biogenesis metallo-sulfur cluster assembly cellular component assembly iron-sulfur cluster assembly negative regulation of programmed cell death regulation of apoptotic process negative regulation of biological process regulation of programmed cell death cell death negative regulation of apoptotic process negative regulation of cellular process apoptotic process programmed cell death regulation of developmental growth regulation of developmental process negative regulation of developmental process negative regulation of organ growth negative regulation of developmental growth negative regulation of growth regulation of organ growth regulation of multicellular organismal process negative regulation of multicellular organismal process [4Fe-4S] cluster assembly response to stimulus response to metal ion response to iron ion response to chemical response to inorganic substance iron incorporation into metallo-sulfur cluster metal incorporation into metallo-sulfur cluster macromolecule modification protein modification process regulation of multicellular organism growth negative regulation of multicellular organism growth multicellular organism growth cell population proliferation positive regulation of cell population proliferation regulation of cell population proliferation regulation of ferrochelatase activity cellular response to stimulus cellular response to oxidative stress response to oxygen-containing compound cellular response to chemical stress cellular response to oxygen-containing compound cellular response to hydrogen peroxide response to reactive oxygen species response to hydrogen peroxide response to oxidative stress cellular response to reactive oxygen species cellular response to chemical stimulus cellular response to stress response to stress signaling negative regulation of signaling apoptotic mitochondrial changes regulation of signal transduction regulation of release of cytochrome c from mitochondria negative regulation of cell communication cell communication negative regulation of release of cytochrome c from mitochondria negative regulation of apoptotic signaling pathway signal transduction mitochondrion organization regulation of signaling negative regulation of cellular component organization negative regulation of response to stimulus negative regulation of signal transduction regulation of organelle organization apoptotic signaling pathway organelle organization release of cytochrome c from mitochondria negative regulation of organelle organization regulation of response to stimulus regulation of mitochondrion organization regulation of cell communication regulation of apoptotic signaling pathway aerobic respiration cellular respiration oxidative phosphorylation energy derivation by oxidation of organic compounds generation of precursor metabolites and energy pigment metabolic process heme metabolic process porphyrin-containing compound biosynthetic process tetrapyrrole metabolic process heme biosynthetic process organonitrogen compound biosynthetic process pigment biosynthetic process organic cyclic compound metabolic process tetrapyrrole biosynthetic process organic cyclic compound biosynthetic process porphyrin-containing compound metabolic process adult walking behavior adult locomotory behavior behavior adult behavior locomotory behavior walking behavior [2Fe-2S] cluster assembly positive regulation of succinate dehydrogenase activity regulation of succinate dehydrogenase activity regulation of oxidoreductase activity positive regulation of oxidoreductase activity multicellular organism development embryo development ending in birth or egg hatching anatomical structure development embryo development muscle cell cellular homeostasis	membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle protein-containing complex cytosol iron-sulfur cluster assembly complex mitochondrial protein-containing complex mitochondrial iron-sulfur cluster assembly complex catalytic complex membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle protein-containing complex cytosol iron-sulfur cluster assembly complex mitochondrial protein-containing complex mitochondrial iron-sulfur cluster assembly complex catalytic complex L-cysteine desulfurase complex membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen organelle lumen
BA [auth h], DA [auth i], FA [auth j], HA [auth k], JA [auth l], BA [auth h], DA [auth i], FA [auth j], HA [auth k], JA [auth l], N [auth a], P [auth b], R [auth c], T [auth d], V [auth e], X [auth f], Z [auth g]	Iron-sulfur cluster assembly enzyme ISCU, mitochondrial	cation binding transition metal ion binding iron ion binding ion binding binding ferrous iron binding metal ion binding small molecule binding identical protein binding protein binding protein homodimerization activity protein dimerization activity iron-sulfur cluster binding 2 iron, 2 sulfur cluster binding cation binding transition metal ion binding iron ion binding ion binding binding ferrous iron binding metal ion binding small molecule binding identical protein binding protein binding protein homodimerization activity protein dimerization activity iron-sulfur cluster binding 2 iron, 2 sulfur cluster binding metal cluster binding molecular adaptor activity zinc ion binding	intracellular iron ion homeostasis inorganic ion homeostasis cellular homeostasis chemical homeostasis homeostatic process intracellular monoatomic cation homeostasis monoatomic ion homeostasis intracellular chemical homeostasis monoatomic cation homeostasis intracellular monoatomic ion homeostasis regulation of lyase activity regulation of molecular function positive regulation of molecular function regulation of catalytic activity intracellular iron ion homeostasis inorganic ion homeostasis cellular homeostasis chemical homeostasis homeostatic process intracellular monoatomic cation homeostasis monoatomic ion homeostasis intracellular chemical homeostasis monoatomic cation homeostasis intracellular monoatomic ion homeostasis regulation of lyase activity regulation of molecular function positive regulation of molecular function regulation of catalytic activity biological regulation positive regulation of catalytic activity positive regulation of aconitate hydratase activity regulation of aconitate hydratase activity positive regulation of lyase activity regulation of localization regulation of cellular process iron import into cell negative regulation of biological process negative regulation of iron ion transmembrane transport negative regulation of transport localization establishment of localization in cell negative regulation of cation transmembrane transport monoatomic cation transport regulation of biological process import across plasma membrane regulation of monoatomic ion transport iron ion transport iron ion transmembrane transport inorganic ion transmembrane transport establishment of localization inorganic cation transmembrane transport negative regulation of iron ion import across plasma membrane transmembrane transport regulation of transmembrane transport import into cell cellular localization transport monoatomic ion transmembrane transport negative regulation of monoatomic ion transport regulation of monoatomic ion transmembrane transport metal ion transport negative regulation of transmembrane transport monoatomic cation transmembrane transport regulation of iron ion transmembrane transport negative regulation of monoatomic ion transmembrane transport negative regulation of cellular process monoatomic ion transport regulation of metal ion transport regulation of iron ion import across plasma membrane iron ion import across plasma membrane negative regulation of iron ion transport regulation of transport regulation of iron ion transport inorganic cation import across plasma membrane regulation of cellular localization inorganic ion import across plasma membrane transition metal ion transport cellular process regulation of monoatomic cation transmembrane transport cellular component biogenesis cellular component organization metallo-sulfur cluster assembly cellular component assembly iron-sulfur cluster assembly cellular component organization or biogenesis aerobic respiration cellular respiration aerobic electron transport chain oxidative phosphorylation energy derivation by oxidation of organic compounds ATP synthesis coupled electron transport positive regulation of biological process electron transport chain regulation of generation of precursor metabolites and energy positive regulation of cellular metabolic process regulation of cellular respiration positive regulation of cellular respiration positive regulation of metabolic process respiratory electron transport chain positive regulation of cellular process cellular metabolic process regulation of cellular metabolic process generation of precursor metabolites and energy mitochondrial electron transport, NADH to ubiquinone regulation of aerobic respiration regulation of mitochondrial electron transport, NADH to ubiquinone positive regulation of mitochondrial electron transport, NADH to ubiquinone metabolic process regulation of metabolic process mitochondrial ATP synthesis coupled electron transport [2Fe-2S] cluster assembly [4Fe-4S] cluster assembly	membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle protein-containing complex cytosol iron-sulfur cluster assembly complex mitochondrial protein-containing complex mitochondrial iron-sulfur cluster assembly complex membrane-enclosed lumen membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle protein-containing complex cytosol iron-sulfur cluster assembly complex mitochondrial protein-containing complex mitochondrial iron-sulfur cluster assembly complex membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen organelle lumen nucleus

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], F [auth N], G [auth O], H [auth P], I [auth Q], J [auth R], K [auth S], L [auth T]	IPR020578	Aminotransferase class-V, pyridoxal-phosphate binding site	Binding Site
A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], F [auth N], G [auth O], H [auth P], I [auth Q], J [auth R], K [auth S], L [auth T]	IPR000192	Aminotransferase class V domain	Domain
A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], F [auth N], G [auth O], H [auth P], I [auth Q], J [auth R], K [auth S], L [auth T]	IPR010240	Cysteine desulfurase IscS	Family
A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], F [auth N], G [auth O], H [auth P], I [auth Q], J [auth R], K [auth S], L [auth T]	IPR016454	Cysteine desulfurase	Family
A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], F [auth N], G [auth O], H [auth P], I [auth Q], J [auth R], K [auth S], L [auth T]	IPR015421	Pyridoxal phosphate-dependent transferase, major domain	Homologous Superfamily
A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], F [auth N], G [auth O], H [auth P], I [auth Q], J [auth R], K [auth S], L [auth T]	IPR015422	Pyridoxal phosphate-dependent transferase, small domain	Homologous Superfamily
A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], F [auth N], G [auth O], H [auth P], I [auth Q], J [auth R], K [auth S], L [auth T]	IPR015424	Pyridoxal phosphate-dependent transferase	Homologous Superfamily
AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], M [auth A], O [auth B], Q [auth C], S [auth D], U [auth E], W [auth F], Y [auth G]	IPR020895	Frataxin conserved site	Conserved Site
AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], M [auth A], O [auth B], Q [auth C], S [auth D], U [auth E], W [auth F], Y [auth G]	IPR036524	Frataxin/CyaY superfamily	Homologous Superfamily
AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], M [auth A], O [auth B], Q [auth C], S [auth D], U [auth E], W [auth F], Y [auth G]	IPR002908	Frataxin/CyaY	Family
AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], M [auth A], O [auth B], Q [auth C], S [auth D], U [auth E], W [auth F], Y [auth G]	IPR017789	Frataxin	Family
BA [auth h], DA [auth i], FA [auth j], HA [auth k], JA [auth l], BA [auth h], DA [auth i], FA [auth j], HA [auth k], JA [auth l], N [auth a], P [auth b], R [auth c], T [auth d], V [auth e], X [auth f], Z [auth g]	IPR011339	Iron-sulfur cluster assembly scaffold protein IscU	Family
BA [auth h], DA [auth i], FA [auth j], HA [auth k], JA [auth l], BA [auth h], DA [auth i], FA [auth j], HA [auth k], JA [auth l], N [auth a], P [auth b], R [auth c], T [auth d], V [auth e], X [auth f], Z [auth g]	IPR002871	NIF system FeS cluster assembly, NifU, N-terminal	Domain

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], A [auth 1], B [auth 2], C [auth 3], D [auth 4], E [auth M], F [auth N], G [auth O], H [auth P], I [auth Q], J [auth R], K [auth S], L [auth T]	Pharos: Q9Y697	Friedreich ataxia Mitochondrial Diseases skin carcinoma large cell medulloblastoma osteosarcoma medulloblastoma ovarian cancer
AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], AA [auth H], CA [auth I], EA [auth J], GA [auth K], IA [auth L], M [auth A], O [auth B], Q [auth C], S [auth D], U [auth E], W [auth F], Y [auth G]	Pharos: Q16595	Friedreich ataxia Friedreich ataxia 1 Ataxias, Hereditary brain disease hypertrophic cardiomyopathy lung cancer osteosarcoma atypical teratoid rhabdoid tumor Charcot-Marie-Tooth disease intellectual disability pancreatic ductal adenocarcinoma autosomal recessive cerebellar ataxia hereditary ataxia large cell medulloblastoma neurodegenerative disease Friedreich ataxia Friedreich ataxia 1 Ataxias, Hereditary brain disease hypertrophic cardiomyopathy lung cancer osteosarcoma atypical teratoid rhabdoid tumor Charcot-Marie-Tooth disease intellectual disability pancreatic ductal adenocarcinoma autosomal recessive cerebellar ataxia hereditary ataxia large cell medulloblastoma neurodegenerative disease tuberculosis
BA [auth h], DA [auth i], FA [auth j], HA [auth k], JA [auth l], BA [auth h], DA [auth i], FA [auth j], HA [auth k], JA [auth l], N [auth a], P [auth b], R [auth c], T [auth d], V [auth e], X [auth f], Z [auth g]	Pharos: Q9H1K1	hereditary myopathy with lactic acidosis due to ISCU deficiency arthrogryposis Friedreich ataxia Mitochondrial Diseases astrocytic tumor ovarian cancer large cell medulloblastoma rhabdomyolysis