ArticleBlood2011
Quaternary organization of GPIb-IX complex and insights into Bernard-Soulier syndrome revealed by the structures of GPIbβ and a GPIbβ/GPIX chimera.
Article in Blood, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 53 citations in OpenAlex.
- New insights of glycoprotein Ib-IX-V complex organization and glycoprotein Ibα in platelet biogenesis.Current opinion in hematology · 2024Review
- Lentiviral gene therapy reverts GPIX expression and phenotype in Bernard-Soulier syndrome type C.Molecular therapy. Nucleic acids · 2023Article
- Establishment of a Bernard-Soulier syndrome model in zebrafish.Haematologica · 2022Article
- Mass spectrometry, data re-analysis, and homology modelling predict posttranslational modifications of leucine-rich alpha-2-glycoprotein as a marker of myelodysplastic syndrome.Cancer biomarkers : section A of Disease markers · 2022Article
- A Novel Mutation inInternational journal of molecular sciences · 2021Article
- Differential regulation of the platelet GPIb-IX complex by anti-GPIbβ antibodies.Journal of thrombosis and haemostasis : JTH · 2021Article
- Structure-function of platelet glycoprotein Ib-IX.Journal of thrombosis and haemostasis : JTH · 2020Review
- A Sardinian founder mutation in glycoprotein Ib platelet subunit beta (GP1BB) that impacts thrombocytopenia.British journal of haematology · 2020Article
- High prevalence of the natural Asn89Asp mutation in the GP1BB gene associated with Bernard-Soulier syndrome in French patients from the genetic isolate of Reunion Island.British journal of haematology · 2020Article
- Unaccompanied mechanosensory domain mediates low expression of glycoprotein Ibα: implications for Bernard-Soulier syndrome.Journal of thrombosis and haemostasis : JTH · 2020Article
- Novel Compound Heterozygous Mutations in Two Families With Bernard-Soulier Syndrome.Frontiers in pediatrics · 2020Article
- Force-Regulated Refolding of the Mechanosensory Domain in the Platelet Glycoprotein Ib-IX Complex.Biophysical journal · 2019Article
- The impact of GPIbα on platelet-targeted FVIII gene therapy in hemophilia A mice with pre-existing anti-FVIII immunity.Journal of thrombosis and haemostasis : JTH · 2019Article
- Shear-Dependent Platelet Aggregation: Mechanisms and Therapeutic Opportunities.Frontiers in cardiovascular medicine · 2019Review
- Refrigeration-Induced Binding of von Willebrand Factor Facilitates Fast Clearance of Refrigerated Platelets.Arteriosclerosis, thrombosis, and vascular biology · 2017Article
- Receptor-mediated cell mechanosensing.Molecular biology of the cell · 2017Review
- Article
- Platelet clearance via shear-induced unfolding of a membrane mechanoreceptor.Nature communications · 2016Article
- Structural basis for the specific inhibition of glycoprotein Ibα shedding by an inhibitory antibody.Scientific reports · 2016Article
- The Transmembrane Domains of β and IX Subunits Mediate the Localization of the Platelet Glycoprotein Ib-IX Complex to the Glycosphingolipid-enriched Membrane Domain.The Journal of biological chemistry · 2015Article
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Authors and funding
7 authors at 2 institutions in 2 countries.
Funding
Abstract
Platelet GPIb-IX receptor complex has 3 subunits GPIbα, GPIbβ, and GPIX, which assemble with a ratio of 1:2:1. Dysfunction in surface expression of the complex leads to Bernard-Soulier syndrome. We have crystallized the GPIbβ ectodomain (GPIbβ(E)) and determined the structure to show a single leucine-rich repeat with N- and C-terminal disulphide-bonded capping regions. The structure of a chimera of GPIbβ(E) and 3 loops (a,b,c) taken from the GPIX ectodomain sequence was also determined. The chimera (GPIbβ(Eabc)), but not GPIbβ(E), forms a tetramer in the crystal, showing a quaternary interface between GPIbβ and GPIX. Central to this interface is residue Tyr106 from GPIbβ, which inserts into a pocket generated by 2 loops (b,c) from GPIX. Mutagenesis studies confirmed this interface as a valid representation of interactions between GPIbβ and GPIX in the full-length complex. Eight GPIbβ missense mutations identified from patients with Bernard-Soulier syndrome were examined for changes to GPIb-IX complex surface expression. Two mutations, A108P and P74R, were found to maintain normal secretion/folding of GPIbβ(E) but were unable to support GPIX surface expression. The close structural proximity of these mutations to Tyr106 and the GPIbβ(E) interface with GPIX indicates they disrupt the quaternary organization of the GPIb-IX complex.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.