[HTML][HTML] Absence of GPIbα is responsible for aberrant membrane development during megakaryocyte maturation: ultrastructural study using a transgenic model
C Poujol, J Ware, B Nieswandt, AT Nurden… - Experimental …, 2002 - Elsevier
OBJECTIVE: The glycoprotein Ib/IX/V complex (GPIb-IX-V) mediates platelet attachment to
von Willebrand factor in exposed subendothelium. Molecular defects in the genes for GPIbα,
GPIbβ, and GPIX give rise to the Bernard-Soulier syndrome, in which thrombocytopenia and
giant platelets suggest that this receptor also is involved in platelet production. To study how
giant platelets are produced in vivo, we used a model of GPIbα-deficient mice (GPIbαnull)
and mice rescued with the human GPIbα transgene (GPIbαnull; hTg). Materials and …
von Willebrand factor in exposed subendothelium. Molecular defects in the genes for GPIbα,
GPIbβ, and GPIX give rise to the Bernard-Soulier syndrome, in which thrombocytopenia and
giant platelets suggest that this receptor also is involved in platelet production. To study how
giant platelets are produced in vivo, we used a model of GPIbα-deficient mice (GPIbαnull)
and mice rescued with the human GPIbα transgene (GPIbαnull; hTg). Materials and …
