Details of Protein

General Information of Protein (ID: PRT00542)
Name Frataxin (FXN)
Synonyms   Click to Show/Hide Synonyms of This Protein
Fxn; Fxn; Frda
Gene Name Fxn Gene ID
14297
UniProt ID
O35943
Family Oxidoreductases (EC 1)
EC Number   EC: 1.16.3.1  (Click to Show/Hide the Complete EC Tree)
Oxidoreductase
Metal ion oxidoreductase
Oxygen acceptor oxidoreductase
EC: 1.16.3.1
  Click to Show/Hide the Molecular/Functional Data (Sequence/Structure/Function) of This Protein
Sequence
MWAFGGRAAVGLLPRTASRASAWVGNPRWREPIVTCGRRGLHVTVNAGATRHAHLNLHYL
QILNIKKQSVCVVHLRNLGTLDNPSSLDETAYERLAEETLDSLAEFFEDLADKPYTLEDY
DVSFGDGVLTIKLGGDLGTYVINKQTPNKQIWLSSPSSGPKRYDWTGKNWVYSHDGVSLH
ELLARELTKALNTKLDLSSLAYSGKGT
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.
Regulatory Network
Full List of Metabolite(s) Regulated by This Protein
      Organo heterocyclic compounds
            Heme Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Fxn
                      Induced Change Heme concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Healthy individual
                      Details It is reported that knockout of Fxn leads to the decrease of heme levels compared with control group.
References
1 Frataxin deficiency alters heme pathway transcripts and decreases mitochondrial heme metabolites in mammalian cells. Hum Mol Genet. 2005 Dec 15;14(24):3787-99.

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