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A model of AIM masking of the A1 domain in VWF. In VWF, the N- and C-terminal sequences flanking the A1 domain, designated as NAIM (orange) and CAIM (celestial blue), respectively, cooperatively form the AIM that binds the A1 domain and blocks its association with GPIbα. Various factors, including ristocetin, type 2B VWD mutations and shear force, may induce VWF binding to GPIbα by destabilizing the AIM/A1 association and/or disrupting AIM.
