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Heat-shock protein gp96/grp94 is an essential chaperone for the platelet glycoprotein Ib-IX-V complex

Staron, Matthew, Wu, Shuang, Hong, Feng, Stojanovic, Aleksandra, Du, Xiaoping, Bona, Robert, Liu, Bei, Li, Zihai·2011·Blood
DOI 10.1182/blood-2011-01-330464
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Abstract

The platelet glycoprotein Ib-IX-V complex (GPIb-IX-IV) is the receptor for VWF and is responsible for VWF-mediated platelet activation and aggregation. Loss of the GPIb-IX-V complex is pathogenic for Bernard-soulier Syndrome (BSS), which is characterized by macrothrombocytopenia and impaired platelet function. It remains unclear how the GPIb-IX-V complex is assembled and whether there is a role for a specific molecular chaperone in the process. In the present study, we report that the assembly of the GPIb-IX-V complex depends critically on a molecular chaperone in the endoplasmic reticulum (ER): gp96 (also known as grp94 and HSP90b1). gp96/grp94 deletion in the murine hematopoietic system results in thrombocytopenia, prolonged bleeding time, and giant platelets that are clinically indistinguishable from human BSS. Loss of gp96/grp94 in vivo and in vitro leads to the concomitant reduction in GPIb-IX complex expression due to ER-associated degradation. We further demonstrate that gp96/grp94 binds selectively to the GPIX subunit, but not to gpIbα or gpIbβ. Therefore, we identify the platelet GPIX subunit of the GPIb-IX-V complex as an obligate and novel client of gp96/grp94.

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Metadata

Authors
Staron, M., Wu, S., Hong, F., Stojanovic, A., Du, X., Bona, R., Liu, B., & Li, Z.
Affiliations
  • Center for Immunotherapy of Cancer and Infectious Diseases, Department of Immunology, University of Connecticut School of Medicine, Farmington, CT;
  • Lea's Foundation Center for Hematological Disorders, University of Connecticut School of Medicine, Farmington, CT; and
  • Department of Pharmacology, University of Illinois at Chicago, Chicago, IL
  • Department of Microbiology & Immunology, Medical University of South Carolina, Charleston, SC;
Venue
Blood
Publisher
American Society of Hematology
Published
2011-06-30
Volume
117
Issue
26
Pages
7136-7144
ISSN
  • 0006-4971
  • 1528-0020
DOI
10.1182/blood-2011-01-330464
Cited by
61
References
1
Language
en

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