The Function of FXN
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.
Protein names
Recommended name:
Frataxin, mitochondrialAlternative name(s):
Friedreich ataxia proteinFxn
i-FXN
56-210
m56-FXN
78-210
d-FXN
m78-FXN
Frataxin(81-210
m81-FXN
- RS7870657 (FXN) ??
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Top Gene-Substance Interactions
FXN Interacts with These Diseases
| Disease | Score |
Substances That Increase FXN
| Substances | Interaction | Organism | Category |
Substances That Decrease FXN
| Substances | Interaction | Organism | Category |
Conditions with Increased Gene Activity
| Condition | Change (log2fold) | Comparison | Species | Experimental variables | Experiment name |
|---|
Conditions with Decreased Gene Activity
| Condition | Change (log2fold) | Comparison | Species | Experimental variables | Experiment name |
|---|
Technical
The following transcription factors affect gene expression:
Tissue specificity:
Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
Gene Pathways:
Molecular Function:
- 2 Iron, 2 Sulfur Cluster Binding
- Ferric Iron Binding
- Ferrous Iron Binding
- Ferroxidase Activity
- Iron Chaperone Activity
- Iron-Sulfur Cluster Binding
Biological Processes:
- Adult Walking Behavior
- Aerobic Respiration
- Cellular Iron Ion Homeostasis
- Cellular Response To Hydrogen Peroxide
- Embryo Development Ending In Birth Or Egg Hatching
- Heme Biosynthetic Process
- Ion Transport
- Iron Incorporation Into Metallo-Sulfur Cluster
- Mitochondrion Organization
- Negative Regulation Of Apoptotic Process
- Negative Regulation Of Multicellular Organism Growth
- Negative Regulation Of Organ Growth
- Negative Regulation Of Release Of Cytochrome C From Mitochondria
- Oxidative Phosphorylation
- Positive Regulation Of Aconitate Hydratase Activity
- Positive Regulation Of Catalytic Activity
- Positive Regulation Of Cell Growth
- Positive Regulation Of Cell Proliferation
- Positive Regulation Of Lyase Activity
- Positive Regulation Of Succinate Dehydrogenase Activity
- Proprioception
- Protein Autoprocessing
- Regulation Of Ferrochelatase Activity
- Response To Iron Ion
- Small Molecule Metabolic Process