Frataxin, Mouse, Control Peptide (FXN)

Artikelnummer: USB-F6100-15
Artikelname: Frataxin, Mouse, Control Peptide (FXN)
Artikelnummer: USB-F6100-15
Hersteller Artikelnummer: F6100-15
Alternativnummer: USB-F6100-15-100
Hersteller: US Biological
Kategorie: Molekularbiologie
Applikation: ELISA
Control Peptide for F6100-05 (antiserum) and F6100-10 (affinity purified antibody). 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 Friedreichs 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 207aa, human 210aa major isoform A, alternatively transcribed minor forms A1, 196aa, and 171aa 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 and Antibody Blocking. Not suitable for use in Western Blot due to low molecular weight. Other applications not tested. Recommended Dilution: Antibody Blocking: 5-10ug per 1ul F6100-05 (antiserum) or per 1ug F6100-10 (affinity purified antibody). ELISA: ~1:100,000. Coat with 50-100ng control peptide/well. Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4C for short-term only. For long-term storage, store at -20C. Aliquots are stable for at least 6 months at -20C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Reinheit: Highly purified
Formulierung: Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.