Disease:Defects in ITGB2 are the cause of leukocyte adhesion deficiency type I (LAD1) [MIM:116920]. LAD1 patients have recurrent bacterial infections and their leukocytes are deficient in a wide range of adhesion-dependent functions. ,Function:Integrin alpha-L/beta-2 is a receptor for ICAM1 , ICAM2 , ICAM3 and ICAM4. Integrins alpha-M/beta-2 and alpha-X/beta-2 are receptors for the iC3b fragment of the third complement component and for fibrinogen. Integrin alpha-X/beta-2 recognizes the sequence G-P-R in fibrinogen alpha-chain. Integrin alpha-M/beta-2 recognizes P1 and P2 peptides of fibrinogen gamma chain. Integrin alpha-M/beta-2 is also a receptor for factor X. Integrin alpha-D/beta-2 is a receptor for ICAM3 and VCAM1. ,online information:ITGB2 mutation db ,PTM:Both Ser-745 and Ser-756 become phosphorylated when T-cells are exposed to phorbol esters. Phosphorylation on Thr-758 (but not on Ser-756) allows interaction with 14-3-3 proteins. ,similarity:Belongs to the integrin beta chain family. ,similarity:Contains 1 VWFA domain. ,subunit:Heterodimer of an alpha and a beta subunit. Beta-2 associates with either alpha-L , alpha-M , alpha-X or alpha-D. Interacts with COPS5 and RANBP9. ,
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