9Z00

Kindlin-3/Integrin Beta3 Cytoplasmic Tail Complex

Summary for 9Z00

• Entry DOI: 10.2210/pdb9z00/pdb
• Descriptor: Fermitin family homolog 3 (2 entities in total)
• Functional Keywords: complex, cell adhesion
• Biological source: Homo sapiens (human); More
• Total number of polymer chains: 2
• Total formula weight: 113156.79

Authors

Xu, Z.,Ma, Y.Q. (deposition date: 2025-10-30, release date: 2026-07-08)

Primary citation

Xu, Z.,Ma, S.,Zhou, Y.,Ma, Y.Q.
Structural basis of kindlin-3 in leukocyte adhesion deficiency III.
J.Thromb.Haemost., 2026

PubMed Abstract: 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.

PubMed: 42362029
DOI: 10.1016/j.jtha.2026.06.021

Experimental method

X-RAY DIFFRACTION (2.19921681269 Å)