You are using an outdated browser. Please upgrade your browser to improve your experience.

Tbio
FXN
Frataxin, mitochondrial

Protein Summary
Description
Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological relevance is unsure as reports are conflicting and the function has only been shown using heterologous overexpression systems. Modulates the RNA-binding activity of ACO1. 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 ...more
Uniprot Accession IDs
Gene Name
Ensembl ID
  • ENST00000396364
  • ENSP00000379650
  • ENSG00000165060
  • ENST00000396366
  • ENSP00000379652
  • ENST00000484259
  • ENSP00000419243
  • ENST00000643639
  • ENSP00000496143

Symbol
  • FRDA
  • X25
  • FA
  • X25
  • CyaY
  • FARR
  • FRDA
Illumination Graph
Knowledge Table
Most Knowledge About
Knowledge Value (0 to 1 scale)
biological process
0.99
biological term
0.9
disease perturbation
0.9
transcription factor binding site profile
0.85
transcription factor perturbation
0.8


IDG Development Level Summary
Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 1564.18   (req: < 5)
Gene RIFs: 170   (req: <= 3)
Antibodies: 547   (req: <= 50)
Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 1564.18   (req: >= 5)
Gene RIFs: 170   (req: > 3)
Antibodies: 547   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 31
Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligand: 0
Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 0