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品种反应性 主要应用 宿主 格式 抗体类型 Chp, H, M, R, Ox, Ca ICC, WB Rb Affinity Purified Polyclonal Antibody 描述 产品目录编号 AB2979 描述 Anti-Kindlin-3 Antibody Alternate Names - kindlin 3
- MIG2-like protein
- UNC-112 related protein 2
- fermitin family homolog 3 (Drosophila)
背景信息 Kindlin-3 is an intracellular protein encoded by the FERMT3 gene and which binds directly to the β chain cytoplasmic tail of integrin in hematopoietic cells. This protein belongs to a family of adhesion plaque proteins, of which there are three members, which act to activate integrins. Kindlin-3 differs from Kindlins-1 and -2 in that its expression is restricted to hematopoietic systems and is especially high in spleen and megakaryocytes. It is a necessary component for integrin activation and for platelet aggregation. Mutations in FERMT3 and consequently to the encoding of Kindlin-3 protein are causal to LAD-I variant syndrome, an uncommon genetic disorder in which integrin adhesive functions on platelets and leukocytes are disrupted. 产品信息 格式 Affinity Purified 控制 - Human lymph node tissue lysate
演示 Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.05% sodium azide. 应用 应用 Detect Kindlin-3 using this Anti-Kindlin-3 Antibody validated for use in WB & IC. 主要应用 - Immunocytochemistry
- Western Blotting
应用说明 Immunocytochemistry Analysis: 1:500 dilution from a previous lot detected Kindlin-3 in RAW264.7 cells. 生物信息 免疫原品种 KLH-conjugated linear peptide corresponding to the FERM domain of human Kindlin-3. 表位 FERM domain 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Rabbit 特异性 The antibody recognizes the FERM domain of Kindlin-3. 品种反应性 ChimpanzeeHumanMouseRatOxCanine Species Reactivity Note Demonstrated to react with human and mouse. Predicted to react with rat, ox, canine, and chimpanzee based on 100% sequence homology. 抗体类型 Polyclonal Antibody Entrez基因编号 - NP_848537
Entrez基因汇总 Kindlins are a small family of proteins that mediate protein-protein interactions involved in integrin activation and thereby have a role in cell adhesion, migration, differentiation, and proliferation. The protein encoded by this gene has a key role in the regulation of hemostasis and thrombosis. This protein may also help maintain the membrane skeleton of erythrocytes. Mutations in this gene cause the autosomal recessive leukocyte adhesion deficiency syndrome-III (LAD-III). Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq]. 基因符号 - kindlin-3
- FERMT3
- KIND3
- Kindlin-3
- MIG-2
- MIG2B
- UNC112C
- URP2
- URP2SF
纯化方法 Affinity Purfied UniProt编号 - Q86UX7
UniProt汇总 FUNCTION: Plays a central role in cell adhesion in hematopoietic cells. Acts by activating the integrin beta-1-3 (ITGB1, ITGB2 and ITGB3). Required for integrin-mediated platelet adhesion and leukocyte adhesion to endothelial cells. Required for activation of integrin beta-2 (ITGB2) in polymorphonuclear granulocytes (PMNs) by similarity. Isoform 2 may act as a repressor of NF-kappa-B and apoptosis.
SUBUNIT STRUCTURE: Interacts with ITGB1, ITGB2 and ITGB3 (via cytoplasmic tails) by similarity.
SUBCELLULAR LOCATION: Cell projection › podosome by similarity. Note: Present in the F-actin surrounding ring structure of podosomes, which are specialized adhesion structures of hematopoietic cells By similarity.
TISSUE SPECIFICTY: Highly expressed in lymph node. Expressed in thymus, spleen and leukocytes. Weakly expressed in placenta, small intestine, stomach, testis and lung. Overexpressed in B-cell malignancies.
DOMAIN: The FERM domain is not correctly detected by PROSITE or Pfam techniques because it contains the insertion of a PH domain.
INVOLVEMENT IN DISEASE: Defects in FERMT3 are the cause of leukocyte adhesion deficiency, type III (LAD3) [MIM:612840]; also called leukocyte adhesion deficiency 1 variant (LAD1v). LAD3 is a rare syndrome characterized by infections without pus formation in the presence of a leukocytosis combined with a Glanzmann-type bleeding disorder, resulting from a hematopoietic defect in integrin activation. Symptoms arise from an inability to activate the integrins expressed on hematopoietic cells, including platelets and leukocytes.
SEQUENCE SIMILARITIES: Belongs to the kindlin family.
Contains 1 FERM domain.
Contains 1 PH domain.产品使用声明 质量保证 Evaluated by Western Blot in human lymph node tissue lysate.
Western Blot Analysis: 0.5 µg/ml of this antibody detected Kindlin-3 in 10 µg of human lymph node tissue lysate.使用声明 - 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.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µg
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