363009
ETFb (Electron Transfer Flavoprotein Beta Polypeptide, ETF-B, MADD, FP585)
Size
£524.00
- SKU:
- 363009
- Purity:
- Purified by immunoaffinity chromatography.
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Rabbit
- Immunogen:
- Recombinant protein corresponding to aa2-255 of human ETFb, expressed in E. coli.
- Format:
- Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
- Extra Details:
- Defects in ETFB are a cause of multiple acyl-CoA dehydrogenase deficiency (MADD); also known as glutaricaciduria IIB and glutaric aciduria IIB (GAIIB). Glutaric aciduria II (GAII) is an autosomal recessively inherited disorder of fatty acid, aa, and choline metabolism. GAIIB results in the excretion not only of glutaric acid but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. It is seen in its most severe form in infancy, with polycystic and dysplastic kidneys, hypoketotic acidosis and hypoglycemia that can lead to death. The milder form can be characterized by recurrent episodes of lasting lethargy or slowly progressive myopathy. Applications: Suitable for use in Western Blot, Immunohistochemistry and Immunocytochemistry/Immunocytochemistry. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. 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