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QuickView for frdA (gene)

Gene Symbol:
FXN Homo sapiens Homo sapiens
Full name:
CyaY, FA, FARR, FRDA, X25, frataxin, mitochondrial, Friedreich ataxia protein
Genomic Location:
Chr 9: 70840164-70878772
This nuclear gene encodes a mitochondrial protein which belongs to the FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA from 8-33 repeats to >90 repeats results in Friedreich ataxia. Alternative splicing results in multiple transcript variants.
Mus musculus Rattus norvegicus Macaca mulatta Schizosaccharomyces pombe Gallus gallus Bos taurus Danio rerio Canis lupus familiaris Pan troglodytes
External Links:
Entrez Gene
Transcription Factor
Binding Sites:
Cytogenetic Map:
chr 9

GO Molecular Function

cofactor binding | ferric iron binding | cation binding | ferrous iron binding | iron chaperone activity | iron ion binding | oxidoreductase activity, oxidizing metal ions | catalytic activity | iron-sulfur cluster binding | ferroxidase activity | ion binding | protein binding | 2 iron, 2 sulfur cluster binding | metallochaperone activity | transition metal ion binding | metal ion binding | oxidoreductase activity

GO Biological Process

positive regulation of lyase activity | proteolysis | sulfur compound metabolic process | regulation of programmed cell death | establishment of localization | negative regulation of cellular process | protein processing | response to metal ion | negative regulation of mitochondrion organization | response to drug | cellular chemical homeostasis | regulation of organelle organization | response to antibiotic | cellular process | localization | cellular homeostasis | negative regulation of signaling | negative regulation of apoptotic process | positive regulation of oxidoreductase activity | positive regulation of catalytic activity | negative regulation of release of cytochrome c from mitochondria | response to inorganic substance | heme metabolic process | porphyrin-containing compound metabolic process | negative regulation of apoptotic signaling pathway | response to hydrogen peroxide | regulation of cellular process | positive regulation of cell growth | regulation of apoptotic process | regulation of metabolic process | organic cyclic compound metabolic process | cellular aromatic compound metabolic process | cellular component organization | response to stimulus | iron-sulfur cluster assembly | oxidoreductase activity, oxidizing metal ions | positive regulation of succinate dehydrogenase activity | response to stress | cellular nitrogen compound biosynthetic process | regulation of cell proliferation | tetrapyrrole biosynthetic process | cellular nitrogen compound metabolic process | cellular response to toxic substance | cofactor metabolic process | cellular component assembly | protein modification process | cellular biosynthetic process | mitochondrion organization | response to iron ion | macromolecule modification | metal ion homeostasis | nitrogen compound metabolic process | regulation of signal transduction | cellular response to antibiotic | regulation of catalytic activity | cellular response to oxidative stress | positive regulation of aconitate hydratase activity | cellular response to stress | cellular ion homeostasis | cofactor biosynthetic process | pigment biosynthetic process | small molecule metabolic process | response to oxidative stress | heme biosynthetic process | biosynthetic process | aromatic compound biosynthetic process | ferroxidase activity | protein maturation | response to toxic substance | regulation of apoptotic signaling pathway | cation homeostasis | cellular response to drug | regulation of mitochondrion organization | cellular cation homeostasis | homeostatic process | positive regulation of cell proliferation | iron ion homeostasis | positive regulation of molecular function | protein metabolic process | regulation of growth | metabolic process | oxidation-reduction process | negative regulation of programmed cell death | cellular response to hydrogen peroxide | chemical homeostasis | cellular response to reactive oxygen species | organic substance metabolic process | pigment metabolic process | response to reactive oxygen species | gene expression | ion homeostasis | cellular iron ion homeostasis | iron incorporation into metallo-sulfur cluster | protein autoprocessing | regulation of ferrochelatase activity | regulation of cell communication | negative regulation of signal transduction | positive regulation of growth | regulation of oxidoreductase activity | cellular protein metabolic process | organelle organization | regulation of cell growth | cellular protein modification process | cellular metal ion homeostasis | heterocycle metabolic process | positive regulation of metabolic process | ion transport | transition metal ion homeostasis | cellular metabolic process | regulation of cell death | oxidoreductase activity | negative regulation of cell death | porphyrin-containing compound biosynthetic process | regulation of cellular component organization | regulation of signaling | macromolecule metabolic process

GO Cellular Component

mitochondrial matrix | intracellular membrane-bounded organelle | cell | cytoplasm | intracellular organelle | protein-containing complex | mitochondrion | L-cysteine desulfurase complex | membrane-bounded organelle | organelle | membrane-enclosed lumen | cytosol | intracellular