BBOX1 (?-butyrobetaine dioxygenase) gene encodes a member of the dioxygenase family. It is found to be expressed in kidney, liver and brain in humans. The gene is mapped to human chromosome 11. The protein contains a catalytic double-stranded ß-helix (DBSH) domain that is conserved among all 2-ketoglutarate oxygenases. It exists as a dimer and contains a small N-terminal domain with a bound Zn ion. Fe(II)-binding residues form a conserved His-X-Asp-X(n)-His triad in the catalytic domain of this protein.
Synonyms: Anti-Gamma- BBH; Anti-Gamma-butyrobetaine dioxygenase; Anti-Gamma-butyrobetaine hydroxylase; Anti-Gamma-butyrobetaine,2- oxoglutarate dioxygenase
Storage: -20C
Application: All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org)and as a result, are supported by the most extensive characterization in the industry. The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
Biochem Physiol Actions: BBOX1 (?-butyrobetaine dioxygenase) gene encodes ?- butyrobetaine hydroxylase that catalyzes the last step in L-carnitine biosynthetic pathway. It hydroxylates ?-butyrobetaine (GBB) to L-carnitine. The final product (carnitine) is an essential component in fatty acid metabolism. It facilitates the transport of activated fatty acids across the mitochondrial membrane during mitochondrial ß-oxidation.
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