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Key Documents

HPA019366

Sigma-Aldrich

Anti-LRBA antibody produced in rabbit

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution, Ab1

Synonyma:

LRBA Antibody - Anti-LRBA antibody produced in rabbit, Lrba Antibody, Anti-Beige-like protein, Anti-CDC4-like protein, Anti-Lipopolysaccharide-responsive and beige-like anchor protein

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About This Item

UNSPSC Code:
12352203
Human Protein Atlas Number:
NACRES:
NA.41

biological source

rabbit

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

technique(s)

immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:200-1:500

immunogen sequence

SVLMVSKYRDILEPQNERHSQSCTETGSENENVSLSEITPAAFSTLTTASVEESESTSSARRRDSGIGEETATGLGSHVEVTPHTAPPGVSAGPDAISEVLSTLSLEVNKSPETKNDRGNDLDTKATPSVSV

UniProt accession no.

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... LRBA(987)

General description

Lrba consists of a BEACH domain situated between a PH-like domain and WD-40 domain.
The gene LRBA (lipopolysaccharide-responsive and beige-like anchor protein) is mapped to human chromosome 4q31.3. It belongs to the BEACH (beige and chediak-higashi)-WD40 (β-transducin) protein family. LRBA is ubiquitously expressed. The protein localizes in the cytoplasm. Upon activation of B cells, LRBA moves to the membrane of trans-Golgi network, lysosomes, endoplasmic reticulum and to the cell surface.

Immunogen

Lipopolysaccharide-responsive and beige-like anchor protein recombinant protein epitope signature tag (PrEST)

Application

Anti-LRBA antibody produced in rabbit has been used:
  • in western blotting
  • fluorescence-activated cell sorting (FACS)
  • basic Local Alignment Search Tool (BLAST) analysis,
  • flow cytometry analysis

Anti-LRBA antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project . Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.

Biochem/physiol Actions

LRBA (lipopolysaccharide-responsive and beige-like anchor protein) participates in endocytosis of ligand-activated receptors. Mutations in LRBA are linked with inflammatory bowel disease (IBD)-like symptoms, autoimmune lymphoproliferative syndrome-like disease and IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked) syndrome.
Lrba interacts with protein kinase A and modulates the intracellular vesicle trafficking to activated receptor complexes. This aids the secretion and membrane deposition of immune effector molecules, like cytotoxic T lymphocyte-associated antigen‐4 (CTLA4). The PH-like domain is critical for membrane association and WD-40 domain helps in ligand binding and signaling.

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 APREST74669

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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Storage Class

10 - Combustible liquids

wgk_germany

WGK 1


Osvědčení o analýze (COA)

Vyhledejte osvědčení Osvědčení o analýze (COA) zadáním čísla šarže/dávky těchto produktů. Čísla šarže a dávky lze nalézt na štítku produktu za slovy „Lot“ nebo „Batch“.

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Dokumenty související s produkty, které jste v minulosti zakoupili, byly za účelem usnadnění shromážděny ve vaší Knihovně dokumentů.

Navštívit knihovnu dokumentů

Clinical, immunological and genetic characteristic of patients with clinical phenotype associated to LRBA-deficiency in Colombia.
Martinez-Jaramillo C, et al.
columbia medica, 50(3), 176-191 (2019)
Rapid flow cytometry-based test for the diagnosis of lipopolysaccharide responsive beige-like anchor (LRBA) deficiency
Gamez-Diaz, Laura et al.
Frontiers in Immunology, 9(3), 720-720 (2018)
LRBA gene deletion in a patient presenting with autoimmunity without hypogammaglobulinemia.
Siobhan O Burns et al.
The Journal of allergy and clinical immunology, 130(6), 1428-1432 (2012-09-18)
Louis-Marie Charbonnier et al.
The Journal of allergy and clinical immunology, 135(1), 217-227 (2014-12-04)
A number of heritable immune dysregulatory diseases result from defects affecting regulatory T (Treg) cell development, function, or both. They include immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, which is caused by mutations in forkhead box P3 (FOXP3), and IPEX-like
The imbalance of circulating T helper subsets and regulatory T cells in patients with LRBA deficiency: correlation with disease severity
Azizi G, et al.
Journal of Cellular Physiology, 233(11), 8767-8777 (2018)

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