Gene Symbol |
FXN
|
Entrez Gene |
505694
|
Alt Symbol |
-
|
Species |
Bovine
|
Gene Type |
protein-coding
|
Description |
frataxin
|
Other Description |
frataxin, mitochondrial
|
Swissprots |
Q05B87
|
Accessions |
DAA26896 Q05B87 BC122620 AAI22621 NM_001080727 NP_001074196
|
Function |
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. Modulates the RNA-binding activity of ACO1 (By similarity). {ECO:0000250}.
|
Subcellular Location |
Cytoplasm {ECO:0000250}. Mitochondrion {ECO:0000250}.
|
Top Pathways |
Porphyrin and chlorophyll metabolism
|