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 ELISA, Western Blot, Immunohistochemistry, Immunocytochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:50-400Immunocytochemistry: 1:50-500Immunohistochemistry (frozen): 1:50-500Immunohistochemistry (paraffin): 1:10-100ELISA: 1:100-1:5000Optimal 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.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes human ETFb.
Isotype：IgG
Calc Applications Abbrev：E IC IHC WB
Calc Crossreactivity：Hu
Immunogen：Recombinant protein corresponding to Ala2-Ile255 of human ETFb, fused to His-Tag and T7-tag at N-terminal expressed in E. coli.
