Intrinsic impaired proplatelet formation and microtubule coil assembly of megakaryocytes in a mouse model of Bernard-Soulier syndrome.

ABSTRACT:

Background

Giant platelets and thrombocytopenia are invariable defects in the Bernard-Soulier syndrome caused by deficiency of the GPIb-V-IX complex, a receptor for von Willebrand factor supporting platelet adhesion to the damaged arterial wall. Various properties of this receptor may be considered potential determinants of the macrothrombocytopenia.

Design and methods

To explore the underlying mechanisms of the disease, megakaryopoiesis was studied in a mouse model deficient in GPIbbeta. Megakaryocytes were initially characterized in situ in the bone marrow of adult mice, after which their capacity to differentiate into proplatelet-bearing cells was evaluated in cultured fetal liver cells.

Results

The number of megakaryocyte progenitors, their differentiation and pro

SUBMITTER: Strassel C 

PROVIDER: S-EPMC2688571 | biostudies-literature | 2009 Jun

REPOSITORIES: biostudies-literature