HPA019366
Anti-LRBA antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution, Ab1
同義詞:
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
推薦產品
生物源
rabbit
共軛
unconjugated
抗體表格
affinity isolated antibody
抗體產品種類
primary antibodies
無性繁殖
polyclonal
產品線
Prestige Antibodies® Powered by Atlas Antibodies
形狀
buffered aqueous glycerol solution
物種活性
human
技術
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:200-1:500
免疫原序列
SVLMVSKYRDILEPQNERHSQSCTETGSENENVSLSEITPAAFSTLTTASVEESESTSSARRRDSGIGEETATGLGSHVEVTPHTAPPGVSAGPDAISEVLSTLSLEVNKSPETKNDRGNDLDTKATPSVSV
UniProt登錄號
運輸包裝
wet ice
儲存溫度
−20°C
目標翻譯後修改
unmodified
基因資訊
human ... LRBA(987)
一般說明
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.
免疫原
Lipopolysaccharide-responsive and beige-like anchor protein recombinant protein epitope signature tag (PrEST)
應用
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.
生化／生理作用
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.
特點和優勢
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:
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.
聯結
Corresponding Antigen APREST74669
外觀
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
法律資訊
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
免責聲明
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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儲存類別代碼
10 - Combustible liquids
水污染物質分類（WGK）
WGK 1
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)
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)
Ulrich Salzer et al.
Arthritis research & therapy, 14(5), 223-223 (2012-10-10)
Common variable immunodeficiency (CVID) describes a heterogeneous subset of hypogammaglobulinemias of unknown etiology. Typically, patients present with recurrent bacterial infections of the respiratory and gastrointestinal tract. A significant proportion of CVID patients develops additional autoimmune, inflammatory or lymphoproliferative complications. CVID
Deniz Cagdas et al.
Journal of clinical immunology, 39(7), 726-738 (2019-08-23)
Autosomal recessively inherited lipopolysaccharide-responsive beige-like anchor (LRBA) protein deficiency was shown to be responsible for different types of inborn errors of immunity, such as common variable immunodeficiency (CVID) and autoimmune lymphoproliferative syndrome (ALPS). The aim of this study was to
Nina Kathrin Serwas et al.
Inflammatory bowel diseases, 21(1), 40-47 (2014-12-06)
Inflammatory bowel diseases (IBDs) denote a heterogeneous group of disorders associated with an imbalance of gut microbiome and the immune system. Importance of the immune system in the gut is endorsed by the presence of IBD-like symptoms in several primary
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