Figure 1.
Clinical phenotype, blood-cell counts, and fucosylation in neutrophils of patient G. (A) Patient G at the age of 26 months. A broad and depressed nasal bridge and divergent strabismus are present. Gastric tube feeding is necessary. (B) Peripheral total white blood cell (WBC) and neutrophil counts of the patient during the first 2 years of life. Total WBC counts were permanently elevated. (C) Fucosylation and selectin ligand expression in neutrophils of the patient. Healthy and patient neutrophils were incubated with the fucose-specific Aleuria aurantia lectin (AAL), anti-Lex, or anti-sLex antibodies, and selectin-IgG (Sel-IgG) chimeric proteins, respectively, before they were analyzed by flow cytometry. Negative controls were secondary antibody only (for AAL), control IgM antibody (for anti-Lex and anti-sLex antibodies) and VE-cadherin-IgG (for selectin-IgG), respectively.