Recombinant Human Platelet glycoprotein Ib alpha chain (GP1BA), partial

1. An autosomal dominant mode of inheritance, a family history of mild bleeding episodes, aggregation pattern in affected individuals together with evidence of mutation occurring in part of the GP1BA gene encoding the leucine-rich repeat region suggest a novel variant causing monoallelic Bernard-Soulier syndrome. PMID: 30332551
2. Combined deficiency of factors V and VIII by chance coinheritance of parahaemophilia and haemophilia A, but not by mutations of either LMAN1 or MCFD2 PMID: 29119711
3. A review of mutations associated with Bernard-Soulier Syndrome and platelet type von Willebrand disease (review). PMID: 28961024
4. ERK5 associates with CKII to play essential roles in GPIb-IX-mediated platelet activation via the PTEN/PI3K/Akt pathway. PMID: 28603902
5. There was no evidence to suggest that polymorphisms of GP VI T13254C and GP Ibalpha VNTR were associated with CAD. PMID: 28607925
6. analysis of an artificial botrocetin that can inhibit the VWF-GPIb interaction PMID: 28071872
7. Loss of the platelet surface receptors GPIbalpha and GPVI in heart failure, CF-VAD and ECMO patients may contribute to ablated platelet adhesion/activation, and limit thrombus formation under high/pathologic shear conditions PMID: 27601054
8. Very low birth weight preterm neonates have increased numbers of platelets interacting with von Willebrand Factor, and increased GPIbalpha expression on the platelet surface PMID: 27416003
9. Data suggest that an aspartate at position 1261 is the most critical residue of VWF N-terminal linker for inhibiting binding of VWF A1 domain to GP1BA on platelets in a model simulating blood flow velocity; network of salt bridges between Asp1261 and rest of VWF A1 domain lock N-terminal linker in place such that binding to GP1BA is reduced. PMID: 28924049
10. Data show that von Willebrand factor (VWF) is first converted from a compact to linear form by flow, and is subsequently activated to bind platelet glycoprotein Ib alpha polypeptide (GPIbalpha) in a tension-dependent manner. PMID: 28831047
11. The >30 nm macroglycopeptide separating the two domains of GPIbalpha transmits force on the VWF-GPIbalpha bond (whose lifetime is prolonged by leucine-rich repeat domain unfolding) to the juxtamembrane mechanosensitive domain to enhance its unfolding, resulting in unfolding cooperativity at an optimal force. PMID: 27434669
12. Meta-analysis found that glycoprotein Ia C807T T allele or the TT genotype, the Ser-allele of HPA-3 and B allele of glycoprotein Ibalpha variable number tandem repeat polymorphisms were associated with increased risk for ischemic stroke. PMID: 28004990
13. Our results suggest that the -5CC genotype in Kozak sequence of GPIb-alpha may be associated with a higher risk of developing arterial ischemia of lower limbs in type 2 diabetes mellitus patients. PMID: 27888791
14. Specific inhibition of GPIbalpha shedding in the stored platelets improves post-transfusion platelet recovery and hemostatic function, providing clear evidence for GPIbalpha shedding as a cause of platelet clearance. PMID: 27417583
15. miR-10a and miR10b regulate the expression of human platelet GP1BA and GP1bb for normal megakaryopoiesis. PMID: 27834869
16. Data indicate that binding of hemoblobin (Hb) to glycoprotein1balpha (GP1balpha) induced platelet activation plays a crucial role in thrombus formation on immobilized von Willebrand factor (VWF) or type I collagen under shear stresses. PMID: 27105433
17. Lateral dimerization of GPIbalpha induced by antibody binding is not sufficient to initiate GPIb-IX signaling and induce platelet clearance. PMID: 26662889
18. GPIb alpha plays a critical role in the co-localization of thrombin and factor XI and the resultant efficient activation of FXI PMID: 12968031
19. Hemoglobin interaction with GP1balpha induces platelet activation and apoptosis: a novel mechanism associated with intravascular hemolysis. PMID: 26341739
20. Both GPIbalpha and PAR4 are required for thrombin-induced reactive oxygen species formation PMID: 26569550
21. Our results reveal the molecular mechanism of collagen-regulated, A1-mediated platelet adhesion enhancement. PMID: 26213126
22. Report no relationship, between polymorphisms of platelet membrane glycoprotein Ibalpha and risk of coronary heart disease in Chinese Han population. PMID: 26191334
23. There is a prevalence of Thr145Met and T(-5)C GP Iba polymorphiams in patients with atherotrombotic stroke due to macroangiopathy. PMID: 26539867
24. Molecular analysis demonstrated a novel homozygous c.800C>G substitution in GP1BA exon 2 leading to a serine 267 Ter stop codon in all 3 siblings PMID: 26044173
25. Whereas VWF-D'D3 is the major regulator of soluble VWF binding to platelet GpIbalpha, both the D'D3-domain and N-terminal peptide regulate platelet translocation and thrombus formation. PMID: 25341886
26. Data indicate that GPIbalpha clustering induced by anti-GPIbalpha N-terminus antibody causes integrin alphaIIbbeta3-dependent platelet aggregation, phagocytosis, and rapid platelet clearance in the liver. PMID: 25231551
27. Data show that force can switch the kinetics of bond formation between A1 domain of von Willebrand factor (VWF) and glycoprotein Ibalpha (GPIbalpha). PMID: 25810255
28. novel mutation that causes von Willebrand disease type 2B is identified in a German family PMID: 24337418
29. The platelet adhesion receptor, the glycoprotein Ib-IX-V complex, not only mediates platelet adhesion but also transmits signals leading to platelet activation, aggregation and secretion PMID: 17414217
30. data indicated that GPIIb-IIIa and GPIb levels are mainly affected by platelet size (MPV) but not by their genetic variations; in some acute coronary syndrome patients, production of large platelets with high GPIIb-IIIa and GPIb contents might be stimulated by elevated thrombopoietin PMID: 23941967
31. Data indicate a G > T in platelet glycoprotein Ib alpha polypeptide (GP1BA) gene, resulting in a Trp to Leu amino acid change at residue 246 (p.W246L), and this mutation was absent in his unaffected mother and also in the 100 controls. PMID: 24474090
32. clone 5G6 showed similar inhibitory potency as a widely used shedding inhibitor GM6001 in both constitutive and induced GPIbalpha shedding in human platelets PMID: 24119228
33. VWF interaction with glycoprotein Ib is modified by polyphosphate PMID: 23006049
34. Our findings indicate that ADAM17 may be a risk factor for ischemic stroke in Chinese and the expression of GPIbalpha can serve as a measure for stroke severity. PMID: 23771674
35. Platelet GP Ib-IX can be considered a multifunctional participant in hemostasis, thrombosis, and the inflammatory cascade. PMID: 24504734
36. Increased mean platelet volume values correlated with increased platelet aggregation activity and enhanced GP IIb-IIIa and GP Ib expression. PMID: 24749250
37. These findings suggest that structural changes, including central GPIbalpha LRR-A1 contact, contribute to VWF affinity regulation. PMID: 24391089
38. genetic association study in population in western India: Data suggest novel mutations in platelet glycoprotein Ib (GP1BA, GP1BB) and GP9 are associated with Bernard-Soulier syndrome in subjects studies; of 12 mutations identified, ten were novel. PMID: 23995613
39. Studies indicate that the platelet-type von Willebrand disease (PT-VWD) is caused by gain-of-function mutations in the platelet GP1BA gene, which codes for the platelet von Willebrand factor (VWF) receptor, GPIbalpha. PMID: 23934752
40. Studies indicate that platelets from Bernard-Soulier syndrome (BSS) are defective in glycoprotein (GP)Ib-IX-V, a platelet-specific adhesion-signaling complex, composed of GPIbalpha disulfide linked to GPIbbeta, and noncovalently associated with GPIX and GPV. PMID: 23929303
41. signaling process through the GPIbalpha cytoplasmic tail required for full platelet activation is defective in BSS variant case II and a length polymorphism of GPIbalpha is associated with a modified level of RIPA heterozygous BSS case I. PMID: 23414566
42. analysis of the localized dynamics-driven affinity regulation mechanism for vWF-GPIbalpha interaction PMID: 23902764
43. These data indicate an important role for the platelet adhesion receptor GPIb-IX in endotoxin-induced thrombosis and thrombocytopenia. PMID: 24051142
44. A considerable fraction of the Jordanian population is resistant to the antiplatelet effect of aspirin, which might be related to GPIba C-5T polymorphism. PMID: 23688555
45. A GP1BA exon 2 fragment spanned the HPA-2 polymorphism and adjacent sequences. PMID: 23750933
46. genotype may predict the development of septic emboli in patients with infective endocarditis PMID: 23611001
47. identify a novel Asp235Tyrmutation in the GP1BA gene of two Iranian patients showing the PT-VWD phenotype who were originally misdiagnosed as type 2B VWD PMID: 23014764
48. GPIX increased the expression of GPIba by promoting the formation of a disulfide bond between GPIba and GPIbb in transfected CHO-K1 cells. PMID: 23143686
49. Data indicate that exposure of von Willebrand factor sites for glycoprotein Ibalpha binding and ADAMTS13 cleavage are coupled. PMID: 22922961
50. The T1255A, Clus1, and DC variants caused increased ristocetin-mediated GPIbalpha binding to VWF. PMID: 22517896

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HGNC: 4439

OMIM: 153670

KEGG: hsa:2811

STRING: 9606.ENSP00000329380

UniGene: Hs.1472