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URP2/Kindlin-3 Rabbit pAb
- 产品详情
- 实验流程
Application 
| IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | Q86UX7 |
| Other Accession | Q86UX7 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 75953 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human URP2 |
| Epitope Specificity | 401-500/667 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the kindlin family. Contains 1 FERM domain. Contains 1 PH domain. |
| 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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. |
| Gene ID | 83706 |
|---|---|
| Other Names | KIND3; MIG-2; MIG2B; UNC112C; URP2; URP2SF; Kindlin3; RGD1310168; URP2_BOVIN; FERMT3; Kindlin-3; Unc-112-related protein 2; URP2_HUMAN; MIG2-like protein; URP2_MOUSE; FERM domain containing kindlin 3; fermitin family homolog 3 (Drosophila); fermitin family member 3; UNC-112 related protein 2 |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. 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. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | FERMT3 |
|---|---|
| Synonyms | KIND3, MIG2B, URP2 |
| Function | Plays a central role in cell adhesion in hematopoietic cells (PubMed:19234463, PubMed:26359933). Acts by activating the integrin beta-1-3 (ITGB1, ITGB2 and ITGB3) (By similarity). Required for integrin-mediated platelet adhesion and leukocyte adhesion to endothelial cells (PubMed:19234460). Required for activation of integrin beta-2 (ITGB2) in polymorphonuclear granulocytes (PMNs) (By similarity). |
| Cellular Location | Cell projection, podosome. Note=Present in the F-actin surrounding ring structure of podosomes, which are specialized adhesion structures of hematopoietic cells |
| Tissue Location | 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. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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