Frataxin (Marker of Friedreich Ataxia) Antibody [FXN/2124]

Additional information

Catalog Number: 2395-MSM4
Reactivity:

Gene:

Host:

In Stock

Trial Size Available

Carrier-Free Available

In Stock
Catalog Number Formulation Size Price
2395-MSM4-P0
Purified Ab with BSA and Azide at 200ug/ml
20ug
$259.00
2395-MSM4-P1
Purified Ab with BSA and Azide at 200ug/ml
100ug
$559.00
2395-MSM4-P1ABX
Purified Ab WITHOUT BSA and Azide at 1.0mg/ml
100ug
$559.00
Flat Rate Domestic: $95 | Orders outside the US - Contact Us for Order Information | Ships next business day
  • IHC
  • WB

Documents

Document Name
Datasheet Download Here
Material Safety Data Sheet Download Here

Applications & Dilutions

Applications Tested Dilutions Protocol Note
Immunohistochemistry (IHC) 1-2ug/ml IHC Protocol 30 min at RT. Staining of formalin-fixed tissues requires heating tissue sections in 10mM Tris with 1mM EDTA, pH 9.0, for 45 min at 95°C followed by cooling at RT for 20 minutes
Western Blot (WB) 2-4ug/ml Western Blot Protocol

Product Properties

Antibody Type
Host
Mouse
Species Reactivity
Clone
FXN/2124
Isotype
IgG2b
Light Chain
Kappa
Cellular Localization
Cytoplasm, Cytosol, Mitochondrion
Gene Name
FXN
Positive Control
HePG2, HL-60 or HEK293 cells. Pancreas and Liver., Panc-2
Immunogen
Recombinant fragment of human Frataxin (FXN) protein (around aa 57-210) (exact sequence is proprietary)
Storage & Stability
Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C. Antibody is stable for 24 months. Non-hazardous. No MSDS required.
Supplied as
200ug/ml of Ab Purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/ml.
Limitations
This antibody is available for research use only and is not approved for use in diagnosis.

Related Antibodies

Other Information

Functions
Key References
Warranty
There are no warranties, expressed or implied, which extend beyond this description. Company is not liable for any personal injury or economic loss resulting from this product.
Entrez Gene ID
SwissProt
Chromosome Location
9q21.11
Mol. Weight of Antigen
14kDa
Alternate Names
Frataxin, mitochondrial, Friedreich ataxia protein, CyaY; d-FXN; FARR; Frataxin mature form; Frataxin (81-210); FRDA; Friedreich ataxia protein; Fxn; i-FXN; m56-FXN; m78-FXN; m81-FXN; X25

Summary

Frataxin is a nuclear-encoded mitochondrial protein implicated in Friedreich’s ataxia (FRDA), a human autosomal recessive neurodegenerative disease. Lack of Frataxin causes iron to accumulate in the mitochondrial matrix suggesting that Frataxin is involved in mitochondrial iron homeostasis and possibly in iron transport. Frataxin has an alpha-beta fold consisting of two helices flanking an antiparallel beta sheet.

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