Annotations: 1EKG

Domain Annotation: SCOP/SCOPe Classification SCOP-e Database Homepage

Chains	Domain Info	Class	Fold	Superfamily	Family	Domain	Species	Provenance Source (Version)
A	d1ekga_	Alpha and beta proteins (a+b)	N domain of copper amine oxidase-like	Frataxin/Nqo15-like	Frataxin-like	C-terminal domain of frataxin	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

Chains	Type	Family Name	Domain Identifier	Family Identifier	Provenance Source (Version)
A	SCOP2 Family	Frataxin-like	8022802	4001566	SCOP2 (2022-06-29)
A	SCOP2 Superfamily	Frataxin/Nqo15-like	8035182	3001840	SCOP2 (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	Frataxin_Cyay	e1ekgA1	A: a+b two layers	X: Type III secretory system chaperone-like	H: Frataxin-like (From Topology)	T: Frataxin-like	F: Frataxin_Cyay	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	3.30.920.10	Alpha Beta	2-Layer Sandwich	Metal Transport, Frataxin	Chain A	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A	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

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A	FRATAXIN	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

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR020895	Frataxin conserved site	Conserved Site
A	IPR036524	Frataxin/CyaY superfamily	Homologous Superfamily
A	IPR002908	Frataxin/CyaY	Family
A	IPR017789	Frataxin	Family

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	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

RCSB PDB Core Operations are funded by the National Science Foundation (DBI-1832184), the US Department of Energy (DE-SC0019749), and the National Cancer Institute, National Institute of Allergy and Infectious Diseases, and National Institute of General Medical Sciences of the National Institutes of Health under grant R01GM133198.