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Els. Buerkel-Rothfuss (Eds. Net Table 1 continued Human disease Autosomal CGD Affected gene Gene productpathogenesis Animal model (species) Similarity to human disease Leucocyte adhesion deficiency syndrome (LAD) LAD1 ITGB2 Integrin β2 (CD18) involved in cell adhesion Dog and cattle with spontaneous mutation in the β2-integrin gene Increased susceptibility to infections, persistent neutrophilia LAD2 - Fucose metabolism deficiency resulting in the absence of selectin ligands Mice L-selectin ko P-selectin ko E- and P-selectin ChédiakHigashi syndrome (CHS) CHS1 Defective formation of lysosomes and cytoplasmic granules Phenotype similar to human reduced chemotactic and bacteriocidal activity of granulocytes, lack of NK cell function (Barbosa et al.

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