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HPA007600

Sigma-Aldrich

Anti-BBOX1 antibody produced in rabbit

enhanced validation

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

Synonym(s):

Anti-Gamma- BBH, Anti-Gamma-butyrobetaine dioxygenase, Anti-Gamma-butyrobetaine hydroxylase, Anti-Gamma-butyrobetaine,2- oxoglutarate dioxygenase

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About This Item

UNSPSC Code:
12352203
Human Protein Atlas Number:
NACRES:
NA.43

biological source

rabbit

Quality Level

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

product line

Prestige Antibodies® Powered by Atlas Antibodies

form

buffered aqueous glycerol solution

species reactivity

human

enhanced validation

orthogonal RNAseq
Learn more about Antibody Enhanced Validation

technique(s)

immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:50-1:200

immunogen sequence

DANNVAYTTGKLSFHTDYPALHHPPGVQLLHCIKQTVTGGDSEIVDGFNVCQKLKKNNPQAFQILSSTFVDFTDIGVDYCDFSVQSKHKIIELDDKGQVVRINFNNATRDTIFDVPVERVQPFYAALKEFVDLMNSKESKLT

UniProt accession no.

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... BBOX1(8424)

General description

BBOX1 (γ-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 β-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.

Immunogen

Gamma-butyrobetaine dioxygenase recombinant protein epitope signature tag (PrEST)

Application

All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project 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. We also provide Prestige Antibodies® protocols and other useful information.

Biochem/physiol Actions

BBOX1 (γ-butyrobetaine dioxygenase) gene encodes γ− butyrobetaine hydroxylase that catalyzes the last step in L-carnitine biosynthetic pathway. It hydroxylates γ-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 β-oxidation.

Features and Benefits

Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:
  • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
  • Protein array of 364 human recombinant protein fragments.

Linkage

Corresponding Antigen APREST71303

Physical form

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide

Legal Information

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Certificates of Analysis (COA)

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Kaspars Tars et al.
Biochemical and biophysical research communications, 398(4), 634-639 (2010-07-06)
Gamma-butyrobetaine hydroxylase (GBBH) is a 2-ketoglutarate-dependent dioxygenase that catalyzes the biosynthesis of l-carnitine by hydroxylation of gamma-butyrobetaine (GBB). l-carnitine is required for the transport of long-chain fatty acids into mitochondria for generating metabolic energy. The only known synthetic inhibitor of
Caroline Rigault et al.
Biochimica et biophysica acta, 1761(12), 1469-1481 (2006-11-18)
Gamma-butyrobetaine hydroxylase (BBOX1) is the enzyme responsible for the biosynthesis of l-carnitine, a key molecule of fatty acid metabolism. This cytosolic dimeric protein belongs to the dioxygenase family. In human, enzyme activity has been detected in kidney, liver and brain.

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