FXN Antibody - #DF6590

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Product: FXN Antibody
Catalog: DF6590
Description: Rabbit polyclonal antibody to FXN
Application: WB IHC
Reactivity: Human, Mouse, Rat
Prediction: Zebrafish, Bovine, Horse, Sheep, Rabbit, Dog, Chicken
Mol.Wt.: 23kDa; 23kD(Calculated).
Uniprot: Q16595
RRID: AB_2838552

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Related Downloads
MS Report
HPLC Report
MSDS
Data Sheet
COA
Protocols
WB Handbook
IHC Protocol
IF/ICC Protocol

Product Info

Source:
Rabbit
Application:
WB 1:500-1:2000, IHC 1:50-1:200
*The optimal dilutions should be determined by the end user.
*Tips:

WB: For western blot detection of denatured protein samples. IHC: For immunohistochemical detection of paraffin sections (IHC-p) or frozen sections (IHC-f) of tissue samples. IF/ICC: For immunofluorescence detection of cell samples. ELISA(peptide): For ELISA detection of antigenic peptide.

Reactivity:
Human,Mouse,Rat
Prediction:
Zebrafish(91%), Bovine(92%), Horse(100%), Sheep(92%), Rabbit(100%), Dog(100%), Chicken(92%)
Clonality:
Polyclonal
Specificity:
FXN Antibody detects endogenous levels of total FXN.
RRID:
AB_2838552
Cite Format: Affinity Biosciences Cat# DF6590, RRID:AB_2838552.
Conjugate:
Unconjugated.
Purification:
The antiserum was purified by peptide affinity chromatography using SulfoLink™ Coupling Resin (Thermo Fisher Scientific).
Storage:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. Store at -20 °C. Stable for 12 months from date of receipt.
Alias:

Fold/Unfold

CyaY; d-FXN; FA; FARR; Frataxin mature form; Frataxin(81-210); FRDA; FRDA_HUMAN; Friedreich ataxia protein; Fxn; i-FXN; m56-FXN; m78-FXN; m81-FXN; MGC57199; X25;

Immunogens

Immunogen:
Uniprot:

Q16595(FRDA_HUMAN) >>Visit HPA database.

Gene(ID):
FXN(2395)
Expression:
Q16595 FRDA_HUMAN:

Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.

Description:
FXN, also named as FRDA, X25, m81-FXN, d-FXN, m78-FXN and i-FXN, belongs to the frataxin family. It promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe2+ to proteins involved in these pathways. FXN may play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe2+ to Fe3+; the oligomeric form but not the monomeric form has in vitro ferroxidase activity. FXN is cleaved to be 4 chains. The mature form of FXN is 14kd or 18kd.
Sequence:
MWTLGRRAVAGLLASPSPAQAQTLTRVPRPAELAPLCGRRGLRTDIDATCTPRRASSNQRGLNQIWNVKKQSVYLMNLRKSGTLGHPGSLDETTYERLAEETLDSLAEFFEDLADKPYTFEDYDVSFGSGVLTVKLGGDLGTYVINKQTPNKQIWLSSPSSGPKRYDWTGKNWVYSHDGVSLHELLAAELTKALKTKLDLSSLAYSGKDA

Predictions

Predictions:

Score>80(red) has high confidence and is suggested to be used for WB detection. *The prediction model is mainly based on the alignment of immunogen sequences, the results are for reference only, not as the basis of quality assurance.

Species
Results
Score
Horse
100
Dog
100
Rabbit
100
Bovine
92
Sheep
92
Chicken
92
Zebrafish
91
Pig
0
Xenopus
0
Model Confidence:
High(score>80) Medium(80>score>50) Low(score<50) No confidence

PTMs - Q16595 As Substrate

Site PTM Type Enzyme
S81 Phosphorylation
T94 Phosphorylation
Y118 Phosphorylation P12931 (SRC)
Y143 Phosphorylation
S160 Phosphorylation
S161 Phosphorylation
K197 Acetylation
K197 Ubiquitination
Y205 Phosphorylation

Research Backgrounds

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.

PTMs:

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 Location:

Mitochondrion. Cytoplasm>Cytosol.
Note: PubMed:18725397 reports localization exclusively in mitochondria.

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionSubcellular location
Tissue Specificity:

Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.

Subunit Structure:

Monomer (probable predominant form). Oligomer. Monomers and polymeric aggregates of >1 MDa have been isolated from mitochondria. A small fraction of heterologous overexpressed recombinant frataxin forms high-molecular wight aggregates that incoroprate iron. Interacts with LYRM4. Interacts with ACO1. Interacts with ISCU isoform 1 and isoform 2. Interacts with FECH; one iron-bound FXN monomer seems to interact with a FECH homodimer. Interacts with SDHA and SDHB. Interacts with ACO2; the interaction is dependent on citrate (By similarity). Interacts with HSPA9.

Family&Domains:

Belongs to the frataxin family.

Research Fields

· Metabolism > Metabolism of cofactors and vitamins > Porphyrin and chlorophyll metabolism.

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