Skip to main content
. Author manuscript; available in PMC: 2013 Feb 5.
Published in final edited form as: Am J Hematol. 2011 Dec 3;87(3):311–313. doi: 10.1002/ajh.22253

Figure 1.

Figure 1

Leukocytes from Patient 2 demonstrated the LAD-Iv/LAD-III phenotype of impaired inside-out signaling of β1 and β2 integrins.

A. PMNs from Patient 2 did not adhere to β2 integrin ligands when activated. PMNs from Patient 2 or a healthy age-matched infant were incubated for 10 min at 37°C in wells coated with bovine serum albumin (BSA), gelatin, or fibrinogen (FB) in the absence of an agonist or with n-formyl-methionyl-phenylalanine (fMLP) (10−7M). Adhesion was quantitated as described in Methods. This figure illustrates results from a single experiment (performed in triplicate). The same pattern was demonstrated in two other experiments in which PMNs from Patient 2 and control subjects were activated with fMLP or PMA (10−7M) (data not shown).

B. Activated PMNs from Patient 2 did not adhere to fibronectin. PMNs from Patient 2 or a control subject were incubated on wells coated with fibronectin in the presence or absence of PMA or fMLP as in panel A. In parallel, PMNs in some wells were treated with the β1 integrin activating antibody TS 2/16 (“act mAb”; 10μg/ml). This figure indicates the pattern of adhesion in a single experiment (performed in triplicate) and is representative of two additional experiments examining primary PMNs from Patient 2.

C. Adhesion of EBV-transformed lymphoblasts from Patient 2 to immobilized ligands was induced by extracellular Mn++ or mAb TS 2/16 (“activating mAb”; see legend to panel B). Lymphoblasts from Patient 2 or a control subject were incubated for 15 min at 37°C in wells coated with fibronectin with buffer alone, or with PMA (10−7M) (performed in triplicate). In parallel wells lymphoblasts were treated with activating mAb, Mn++ (1μM), or Mn++ together with a blocking anti- β1 mAb (mAb p4C10, 10 μg/ml). A similar pattern was seen in a second experiment. In additional experiments, extracellular Mn++ induced adhesion of lymphoblasts from Patient 2 to immobilized ICAM-1, a ligand for β2 integrins; this was inhibited by a blocking anti- β2 mAb (mAb 60.3; 10μg/ml) (N=2, data not shown).