The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Additional notesBest use within three months from the date of receipt of this protein.
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Preparation and Storage
Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.79% Tris HCl, 0.31% Glutathione
Fermitin family homolog 3
Fermitin family member 3
MIG2 like protein
Unc 112 related protein 2
Unc-112-related protein 2
FunctionPlays 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.
Tissue specificityHighly 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.
Involvement in diseaseDefects 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.
Sequence similaritiesBelongs to the kindlin family. Contains 1 FERM domain. Contains 1 PH domain.
DomainThe FERM domain is not correctly detected by PROSITE or Pfam techniques because it contains the insertion of a PH domain.
Cellular localizationCell projection > podosome. Present in the F-actin surrounding ring structure of podosomes, which are specialized adhesion structures of hematopoietic cells.