Elemental iron is required for a variety of normal cellular functions and vital for proper growth and development.
However, natural iron is quite insoluble and excess iron is harmful, since it can catalyze the formation of potentially damaging reactive oxygen species.
Humans also have very limited capacity to excrete iron.
Therefore, cells have developed mechanisms to improve solubility of iron and to control intracellular iron levels at the point of absorption in the intestine and other tissue.
Several proteins including Ferritin, transferrin (Tf), transferrin receptors (TfRs), and iron regulatory proteins (IRPs), iron transporter (NRMAP2/DMT1/DCT1) etc.
play a key role in iron metabolism.
Some genes involved in iron-metabolism are associated with genetic disorders such as Friedreich's Ataxia (Frataxin), genetic hemochromatosis (HFE), and Sex-linked anemia (Hephaestin).
Friedrich ataxia (FA), a degenerative disease characterized by progressive ataxia and hypertrophic cardiomyopathy leading to premature death, is cause by a deficiency in frataxin (Fxn).
Fxn (mouse 207 aa, human 210 aa major isoform A; alternatively transcribed minor forms A1, 196 aa, and 171-aa isoform B) is a nuclear-encoded and highly conserved mitochondrial membrane protein.
Fxn is expressed in tissues with highly metabolic activity, such as heart, liver, and brown fat.
In FA, Fxn levels are greatly reduced due to inhibition of its transcription by expansion of an intronic GAA repeat.
Defects in yeast Fxn produce mitochondria iron-overload.
It is suggested that Fxn could play a role in mitochondrial iron storage.
Applications:Suitable for use in ELISA.
Western Blot, though not tested, may potentially be used as an application.
Other applications not tested.
Recommended Dilutions:Western Blot: 1-10ug/ml using ECL
ELISA: 1:100,000 using 50-100ng control peptide/wellOptimal dilutions to be determined by the researcher.
Control Peptide:F6100-15: Frataxin, Mouse, Control Peptide (FXN)
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ug
Host：rabbit
Source Antibody：mouse
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.5, 0.1% BSA.
Specificity：Recognizes mouse Frataxin (FXN).
Isotype：IgG
Calc Applications Abbrev：E
Calc Crossreactivity：Mo
Immunogen：A synthetic peptide corresponding to 20aa within the C-terminus of mouse FXN, conjugated to KLH. Species Sequence Homology: human, 94%
