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Key Documents

HPA023597

Sigma-Aldrich

Anti-LRBA antibody produced in rabbit

enhanced validation

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution, Ab2

Synonyme(s) :

Anti-Beige-like protein, Anti-CDC4-like protein, Anti-Lipopolysaccharide-responsive and beige-like anchor protein

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About This Item

Code UNSPSC :
12352203
Numéro HPA (Human Protein Atlas):
Nomenclature NACRES :
NA.41

Source biologique

rabbit

Niveau de qualité

Conjugué

unconjugated

Forme d'anticorps

affinity isolated antibody

Type de produit anticorps

primary antibodies

Clone

polyclonal

Gamme de produits

Prestige Antibodies® Powered by Atlas Antibodies

Forme

buffered aqueous glycerol solution

Espèces réactives

human

Validation améliorée

RNAi knockdown
Learn more about Antibody Enhanced Validation

Technique(s)

immunohistochemistry: 1:1000- 1:2500

Séquence immunogène

GGWRVWVDTLSITHSKVTFEIHKENLANIFREQQGKVDEEIGLCSSTSVQAASGIRRDINVSVGSQQPDTKDSPVCPHFTTNGNENSSIEKTSSLESASNIELQTTNTSYEEMKAEQENQELPDEGTLEETL

Numéro d'accès UniProt

Conditions d'expédition

wet ice

Température de stockage

−20°C

Modification post-traductionnelle de la cible

unmodified

Informations sur le gène

human ... LRBA(987)

Description générale

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.

Immunogène

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.
All Prestige Antibodies®Powered by Atlas Antibodies 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. We also provide Prestige Antibodies® protocols and other useful information.
Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.
Western Blotting (1 paper)

Actions biochimiques/physiologiques

The protein encoded by the gene 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.

Caractéristiques et avantages

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.

Liaison

Corresponding Antigen APREST74668

Forme physique

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

Informations légales

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

Clause de non-responsabilité

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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Code de la classe de stockage

10 - Combustible liquids

Classe de danger pour l'eau (WGK)

WGK 1


Certificats d'analyse (COA)

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Retrouvez la documentation relative aux produits que vous avez récemment achetés dans la Bibliothèque de documents.

Consulter la Bibliothèque de documents

Atypical Manifestation of LPS-Responsive Beige-Like Anchor Deficiency Syndrome as an Autoimmune Endocrine Disorder without Enteropathy and Immunodeficiency.
Bakhtiar S
Frontiers in pediatrics, 4 (2016)
Shahrzad Bakhtiar et al.
Frontiers in pediatrics, 4, 98-98 (2016-09-30)
Monogenic primary immunodeficiency syndromes can affect one or more endocrine organs by autoimmunity during childhood. Clinical manifestations include type 1 diabetes mellitus, hypothyroidism, adrenal insufficiency, and vitiligo. Lipopolysaccharide (LPS)-responsive beige-like anchor protein (LRBA) deficiency was described in 2012 as a
Wen-Juan Tang et al.
World journal of clinical cases, 9(21), 5873-5888 (2021-08-10)
Patients with lipopolysaccharide (LPS)-responsive beige-like anchor protein (LRBA) deficiency have a variety of clinical symptoms, but there is no apparent genotype-phenotype correlation, and patients carrying the same mutations may have different phenotypes. Therefore, it is not easy for doctors to
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
Iman Hawari et al.
Biology, 11(4) (2022-04-24)
The scope of this study is to show that DM in a LRBA-deficient patient with a stop codon mutation (c.3999 G > A) was not mediated through autoimmunity. We have evaluated the ability of the proband's T cells to be

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