产品详情
英文名称 URP2/Kindlin-3
中文名称 整合素结合性信号分子抗体
别 名 Fermitin family homolog 3; Fermitin family member 3; FERMT3; Kind3; Kindlin 3; Kindlin-3; Kindlin3; MGC10966; MIG 2; MIG2 like protein; MIG2-like protein; MIG2B; Unc 112 related protein 2; Unc-112-related protein 2; UNC112C; URP2_HUMAN; URP2SF.
整合素结合性信号分子抗体
说 明 书 0.1ml 0.2ml
研究领域 肿瘤 细胞生物 信号转导 细胞粘附分子
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Pig, Cow, Horse,
产品应用 WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 76kDa
细胞定位 细胞膜
性 状 Lyophilized or Liquid
浓 度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human URP2
亚 型 IgG
纯化方法 affinity purified by Protein A
储 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
整合素结合性信号分子抗体 产品介绍 background:
Kindlin-3 is a 667 amino acid protein that localizes to both the cell membrane and the cytoplasm and contains one PH domain and one FERM domain. Expressed at high levels in lymph node tissue and at lower levels in spleen, thymus, stomach, placenta, lung, testis and small intestine, Kindlin-3 is thought to be involved in cell adhesion events and may play a role in apoptosis. Kindlin-3 is overexpressed in B-cell malignancies, suggesting that, via its ability to affect cell adhesion, Kindlin-3 may participate in tumor transformation and metastasis. Two isforms of Kindlin-3, designated short and long, exist due to alternative splicing events.
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).
Isoform 2 may act as a repressor of NF-kappa-B and apoptosis.
Subcellular Location:
Cell projection; podosome. Present in the F-actin surrounding ring structure of podosomes, which are specialized adhesion structures of hematopoietic cells.
Tissue Specificity:
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.
DISEASE:
Defects in FERMT3 are the cause of leukocyte adhesion deficiency type 3 (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.
Similarity:
Belongs to the kindlin family.
Contains 1 FERM domain.
Contains 1 PH domain.
Gene ID:
83706
Database links:
Entrez Gene: 83706 Human
Entrez Gene: 525159 Cow
Entrez Gene: 108101 Mouse
Entrez Gene: 309186 Rat
Omim: 607901 Human
SwissProt: Q32LP0 Cow
SwissProt: Q86UX7 Human
SwissProt: Q8K1B8 Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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