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Thromb Haemost 2000; 84(02): 312-318
DOI: 10.1055/s-0037-1614013
Review Article
Schattauer GmbH

Ultrastructural Analysis of Megakaryocytes in GPV Knockout Mice

Christel Poujol
2   From UMR 5533 CNRS, Hôpital Cardiologique, Pessac, France
,
Vanitha Ramakrishnan
1   Cor Therapeutics, South San Francisco, California, USA
,
Francis DeGuzman
1   Cor Therapeutics, South San Francisco, California, USA
,
Alan T. Nurden
2   From UMR 5533 CNRS, Hôpital Cardiologique, Pessac, France
,
David R. Phillips
1   Cor Therapeutics, South San Francisco, California, USA
,
Paquita Nurden
2   From UMR 5533 CNRS, Hôpital Cardiologique, Pessac, France
› Author AffiliationsC.P. was the recipient of a post-doctoral fellowship from the Institut Electricité Santé.
Further Information

Publication History

Received 06 January 2000

Accepted after revision 21 March 2000

Publication Date:
14 December 2017 (online)

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Summary

Lesions in the genes for GPIbα, GPIbβ or GPIX result in a bleeding diathesis, the Bernard-Soulier syndrome (BSS), which associates a platelet adhesion defect with thrombocytopenia, giant platelets and abnormal megakaryocytes (MK). The role of GPV, also absent in BSS, was recently addressed by gene targeting in mice. While a negative modulator function for GPV on thrombin-induced platelet responses was found in one model, the absence of GP V had no effect on GPIb-IX expression or platelet adhesion. Our study extends previous results and reports that electron microscopy of bone marrow from the GPV knockout mice revealed a normal MK ultrastructure and development of the demarcation membrane system (DMS). There was a usual presence of MK fragments in the bone marrow vascular sinus. Immunogold labelling of MK from the knockout mice showed a normal distribution of GPIb-IX in the DMS and on the cell surface. The distribution of fibrinogen, vWF and P-selectin was unchanged with, interestingly, P-selectin also localised within the DMS in both situations. Thus GPV is not crucial to MK development and platelet production, consistent with the fact that no mutation in the GPV gene has as yet been described in BSS.

Key words

GPV knockout mice - GPIb-IX - megakaryocytes - electron microscopy
 

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