F6100
Frataxin (FXN)
Size
£960.00
- SKU:
- F6100
- Purity:
- Ascites
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Mouse
- Immunogen:
- TrpE-Frataxin full length. Epitope: Maps to the first part of exon 4 of frataxin.
- Clone:
- 2Q54 (1G2)
- Format:
- Supplied as a liquid in PBS. No preservatives added.
- Extra Details:
- Frataxin is a monomeric mitochondrial protein that is believed to be involved in iron homeostasis through an unknown mechanism. Expression of frataxin is highest in tissue rich with mitochondria including liver, heart, and skeletal muscle. Frataxin is expressed as a 30kD precursor that is processed within in the mitochondria in two steps catalysed by the mitochondrial processing peptidase to yield the mature protein. The first step involves cleavage of the first 41 N-terminal amino acids by MPP yielding a transient intermediate of approximately 20kD (aa 42-210). Further cleavage of the N-terminus of this 20kD intermediate by MPP results in the mature 18kD frataxin protein (aa 56-210). Defects in the gene encoding frataxin are implicated as the cause of Friedreich’s ataxia, an autosomal recessive, progressive degenerative disease. In the majority of cases of Friedreich’s ataxia, there is an expansion of a trinucleotide repeat in the first intron of the gene encoding frataxin resulting in a marked decrease in frataxin expression, perhaps due to the formation of some unusual yet stable DNA structure that interferes with transcription. This reduction in frataxin expression results in the accumulation of unchelated iron in the mitochondria, inhibition of mitochondrial iron-sulfer proteins, and iron mediated oxidative stress. Applications: Suitable for use in Western Blot, Immunofluorescence and ELISA. Other applications not tested. Recommended Dilution: Western Blot (Natural and recombinant protein): 1:2500 Immunofluorescence: 1:50-1:500 Fixation of cells in ice cold acetone or 4% paraformaldehyde is recommended. Due to subcellular localization of frataxin in the mitochondria, cells should be permeabilized in the presence of detergent prior to incubation with primary antibody. Optimal dilutions to be determined by the researcher. Recommended Positive Control: Liver, heart or skeletal muscle. 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.
- Shipping Conditions:
- Blue Ice