Abstract:
:Glycoprotein (GP) Ibalpha, a member of the leucine-rich repeat (LRR) protein family, mediates platelet adhesion to immobilized von Willebrand factor (VWF). We investigated the role in VWF binding of charged residues in the LRR region of GP Ibalpha that are conserved in human, canine, and murine proteins. Substitution of His86 with either Ala or Glu resulted in a gain of VWF-binding function as judged by increased VWF binding in the presence of the modulators ristocetin and botrocetin and by enhanced adhesion of Chinese hamster ovary (CHO) cells expressing the mutant GP Ibalpha to immobilized VWF under conditions of flow. This is the first report of a gain-of-function phenotype resulting from mutations in the LRR region of GP Ibalpha. Because His86 is 2 nm away from the region of GP Ibalpha with the largest surface of contact with VWF, the data suggest that the LRRs regulate GP Ibalpha affinity for VWF allosterically.
journal_name
Bloodjournal_title
Bloodauthors
Peng Y,Shrimpton CN,Dong JF,López JAdoi
10.1182/blood-2005-02-0514subject
Has Abstractpub_date
2005-09-15 00:00:00pages
1982-7issue
6eissn
0006-4971issn
1528-0020pii
2005-02-0514journal_volume
106pub_type
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