URP2/kindlin-3, Polyclonal Antibody

FERMT3; fermitin family homolog 3 (Drosophila); KIND3; MGC10966; MIG-2; MIG2B; UNC112C; URP2; URP2SF; UNC-112 related protein 2; fermitin family homolog 3; kindlin 3; kindlin-3

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Goat

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

Aliquot and store at -20 degree C. Minimize freezing and thawing.

Manufactured in an ISO 9001:2008 Certified Laboratory.

Small volumes of anti-FERMT3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Peptide ELISA (EIA), Western Blot (WB)

Peptide ELISA: Antibody detection limit dilution 1: 2000.
Western Blot: Approx 75kDa band observed in Human Peripheral Blood Mononucleocytes lysates (calculated MW of 75.4kDa according to NP_113659.3). Recommended concentration: 1-3ug/ml.

(1ug/ml) staining of Human Peripheral Blood Mononucleocytes lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.

fermitin family member 3

fermitin family homolog 3

Fermitin family homolog 3

KIND3; MIG2B; URP2  [Similar Products]

URP2_HUMAN

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, Jan 2010]

kindlin-3: 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). Defects in FERMT3 are the cause of leukocyte adhesion deficiency type 3 (LAD3); 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. Belongs to the kindlin family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Cell adhesion

Chromosomal Location of Human Ortholog: 11q13.1

Cellular Component: cell junction; cell projection; membrane; podosome

Molecular Function: integrin binding

Biological Process: integrin activation; integrin-mediated signaling pathway; leukocyte adhesion; positive regulation of cell migration; regulation of cell-cell adhesion mediated by integrin

Disease: Leukocyte Adhesion Deficiency, Type Iii

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