BBOX1 (butyrobetaine (y), 2-oxoglutarate dioxygenase 1), also known as BBH, BBOX, G-BBH (y-butyrobetaine hydroxylase) or y-BBH, is a member of the y-BBH/TMLD family of proteins.
Localizing to the cytoplasm and predominantly expressed in kidney, BBOX1 plays a role in the biosynthesis of amines, polyamines and carnitines.
More specifically, BBOX1 catalyzes the hydroxylation of y-butyrobetaine to L-carnitine, a reaction that requires iron and ascorbate as cofactors.
This is the last of five steps comprising the L-carnitine biosynthesis pathway and it is important for the proper transport of activated fatty acids across the mitochondrial membrane.
Coupled to this reaction, BBOX1 simultaneously catalyzes the oxidative decarboxylation of alpha-ketoglutarate to succinate.
Applications:Suitable for use in Western Blot, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal 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：50ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.1% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of BBOX1. Species Crossreactivity: Human
