Recombinant Human Frataxin protein (Tagged) is a Human Full Length protein, in the 1 to 210 aa range, expressed in Escherichia coli, with >85% purity and suitable for SDS-PAGE.
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Key facts
- Purity
>85% SDS-PAGE
- Expression system
Escherichia coli
- Tags
His tag N-Terminus
- Applications
SDS-PAGE
- Biologically active
No
Amino acid sequence
M W T L G R R A V A G L L A S P S P A Q A Q T L T R V P R P A E L A P L C G R R G L R T D I D A T C T P R R A S S N Q R G L N Q I W N V K K Q S V Y L M N L R K S G T L G H P G S L D E T T Y E R L A E E T L D S L A E F F E D L A D K P Y T F E D Y D V S F G S G V L T V K L G G D L G T Y V I N K Q T P N K Q I W L S S P S S G P K R Y D W T G K N W V Y S H D G V S L H E L L A A E L T K A L K T K L D L S S L A Y S G K D A
Reactivity data
| Application | Reactivity | Dilution info | Notes |
|---|---|---|---|
Application SDS-PAGE | Reactivity Reacts | Dilution info - | Notes - |
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Target data
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; 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.
Alternative names
Friedreich ataxia protein, Fxn, FXN, FRDA, X25
Recommended products
Recombinant Human Frataxin protein (Tagged) is a Human Full Length protein, in the 1 to 210 aa range, expressed in Escherichia coli, with >85% purity and suitable for SDS-PAGE.
Alternative names
Friedreich ataxia protein, Fxn, FXN, FRDA, X25
Key facts
- Purity
>85% SDS-PAGE
- Expression system
Escherichia coli
- Tags
His tag N-Terminus
- Applications
SDS-PAGE
- Biologically active
No
- Accession
- Q16595-1
- Animal free
No
- Species
Human
- Concentration
- Storage buffer
pH: 7.2 - 7.4
Constituents: Tris buffer, 50% Glycerol (glycerin, glycerine)
Sequence info
Amino acid sequence
M W T L G R R A V A G L L A S P S P A Q A Q T L T R V P R P A E L A P L C G R R G L R T D I D A T C T P R R A S S N Q R G L N Q I W N V K K Q S V Y L M N L R K S G T L G H P G S L D E T T Y E R L A E E T L D S L A E F F E D L A D K P Y T F E D Y D V S F G S G V L T V K L G G D L G T Y V I N K Q T P N K Q I W L S S P S S G P K R Y D W T G K N W V Y S H D G V S L H E L L A A E L T K A L K T K L D L S S L A Y S G K D A
- Accession
- Protein length
Full Length
- Predicted molecular weight
28.1 kDa
- Amino acids
1 to 210
- Nature
Recombinant
- Tags
His tag N-Terminus
Specifications
- Form
Liquid
General info
- 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; 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.
- Sequence similarities
Belongs to the frataxin family.
- Post-translational modifications
Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to yield frataxin mature form (frataxin(81-210)) which is the predominant form. The additional forms, frataxin(56-210) and frataxin(78-210), seem to be produced when the normal maturation process is impaired; their physiological relevance is unsure.
- Subcellular localisation
Mitochondrion
Storage
- Shipped at conditions
Blue Ice
- Appropriate long-term storage conditions
-20°C
- Storage information
Avoid freeze / thaw cycle
Supplementary info
- Activity summary
Frataxin often known by the alternate name FXN is a mitochondrial protein with a mass of approximately 21000 Dalton. It is expressed mainly in tissues with high energy demands like the heart liver and pancreas. Frataxin plays an important role in iron-sulfur cluster assembly which is essential for various cellular processes. The protein is a part of mitochondria where it regulates iron homeostasis and prevents oxidative damage by minimizing iron-induced free radical generation.
- Biological function summary
Several cellular processes depend on the correct function of this protein. Frataxin assists in forming iron-sulfur clusters acting within a multiprotein complex in the mitochondria. The complex includes proteins such as ISCU which are involved in the assembly and repair of iron-sulfur clusters. These clusters are necessary for supporting mitochondrial electron transport and other fundamental metabolic pathways that require iron-sulfur dependencies.
- Pathways
Frataxin's involvement extensively affects the mitochondrial respiratory chain and the mitochondrial biogenesis process. It plays a role in the electron transport chain by stabilizing iron-sulfur-containing complexes. NAB is one associated protein that interacts closely within these pathways sharing a connection through iron-sulfur cluster transportation and assembly systems. Efficient function of these pathways ensures a proper energetic output of cells.
- Associated diseases and disorders
Frataxin mutations are directly linked to Friedreich's ataxia a neurodegenerative disease causing progressive damage to the nervous system. The deficiency or dysfunction in frataxin causes accumulation of iron in mitochondria leading to increased oxidative stress. Another related disorder includes heart disease which emerges due to the same oxidative stress pathway. Proteins such as Nfs1 are also involved sharing the responsibility with frataxin in scavenging excess iron protecting against related tissue damage.
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SDS-PAGE - Recombinant Human Frataxin protein (Tagged) (ab238218)
ab238218 analyzed by (Tris-Glycine gel) discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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