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Structural basis of kindlin-3 in leukocyte adhesion deficiency III
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DOI：10.1016/j.jtha.2026.06.021.png)
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Abstract
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Leukocyte adhesion deficiency III (LAD-III) is caused by impaired integrin activation in hematopoietic cells due to mutations in FERMT3, which encodes kindlin-3, an FERM domain–containing adaptor, and is an essential integrin coactivator that binds to the cytoplasmic tails (CTs) of integrin β subunits. Defective kindlin-3 impairs integrin activation in both leukocytes and platelets, leading to recurrent infections and severe bleeding. More than 30 pathogenic FERMT3 mutations have been identified, including 7 missense variants that localize to the FERM domain, specifically within the F1 (L102P, E138K, W229C, and K252N), F2 (L274R), and F3 (L574P and Q595P) subdomains.
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