The Tetraspanin CD9 Localizes to Platelet-Platelet Contacts and Regulates Thrombus Stability

Hill, Sarah Kathleen

doi:10.21007/etd.cghs.2008.0138

Cited by 2 publications

(3 citation statements)

References 224 publications

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“…It is hypothesized that CD9 modulates integrin αIIbβ3, the major platelet integrin involved in thrombus stability. CD9 also regulates platelet activation and aggregation and cell adhesion [23].…”

Section: Discussionmentioning

confidence: 99%

A GP1BA Variant in a Czech Family with Monoallelic Bernard-Soulier Syndrome

Skalnı́ková

Kozubík

Trizuljak

et al. 2022

IJMS

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Bernard-Soulier syndrome (BSS) is a rare inherited disorder characterized by unusually large platelets, low platelet count, and prolonged bleeding time. BSS is usually inherited in an autosomal recessive (AR) mode of inheritance due to a deficiency of the GPIb-IX-V complex also known as the von Willebrand factor (VWF) receptor. We investigated a family with macrothrombocytopenia, a mild bleeding tendency, slightly lowered platelet aggregation tests, and suspected autosomal dominant (AD) inheritance. We have detected a heterozygous GP1BA likely pathogenic variant, causing monoallelic BSS. A germline GP1BA gene variant (NM_000173:c.98G > A:p.C33Y), segregating with the macrothrombocytopenia, was detected by whole-exome sequencing. In silico analysis of the protein structure of the novel GPIbα variant revealed a potential structural defect, which could impact proper protein folding and subsequent binding to VWF. Flow cytometry, immunoblot, and electron microscopy demonstrated further differences between p.C33Y GP1BA carriers and healthy controls. Here, we provide a detailed insight into its clinical presentation and phenotype. Moreover, the here described case first presents an mBSS patient with two previous ischemic strokes.

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Section: Discussionmentioning

confidence: 99%

A GP1BA Variant in a Czech Family with Monoallelic Bernard-Soulier Syndrome

Skalnı́ková

Kozubík

Trizuljak

et al. 2022

IJMS

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“…104 Recent studies performed using monovalent Fab fragments of CD9 mAbs reveal that engagement of CD9 with Fab promotes aggregate stability and enhanced fibrinogen binding to GPIIb/IIIa under threshold concentrations of agonists. 105 Similarly, recombinant protein corresponding to the CD9 EC2 domain inhibits low-dose agonist induced platelet aggregation (J. Kotha et al, unpublished observations). Scanning electron microscopy with immunogold labelling with CD9 mAb suggest that CD9 is enriched at the regions of platelet-platelet contacts and is localized with the GPIIb/IIIa in the alpha granules and pseudopodial process of activated platelets.…”

Section: Cd9mentioning

confidence: 97%

“…106 Confocal laser scanning microscopy studies on platelets at various stages of spreading reveal that in addition to high-density localization of CD9 to platelet-platelet contact sites, filopodial extensions show markedly enhanced CD9 staining. 105 Significance of CD9 in platelet function and haemostasis has also been studied using CD9 KO mice model. Despite being the most abundant tetraspanin on platelets, CD9 deficiency does not appear to alter agonist-induced platelet aggregation or expression of the platelet activation marker, P-selectin (F. Lanza, personal communication).…”

Section: Cd9mentioning

confidence: 99%

Tetraspanins and vascular functions

Zhang

Kotha

Jennings

et al. 2009

Cardiovascular Research

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Tetraspanins are multiple membrane-spanning proteins that likely function as the organizers of membrane microdomains. Tetraspanins associate with other membrane-bound molecules such as cell-adhesion proteins, growth factor receptors, and Ig superfamily members and regulate key cellular processes such as adhesion, migration, and fusion. Tetraspanins are widely expressed in vascular and haematopoietic cells and are involved in both physiological and pathological processes related to angiogenesis, vascular injury, thrombosis, and haemostasis. A wide body of evidence suggests that tetraspanins directly regulate the development and functions of the vascular system and the pathogenesis of vascular diseases. This article reviews current understanding of the roles of tetraspanins in vascular functions.

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The Tetraspanin CD9 Localizes to Platelet-Platelet Contacts and Regulates Thrombus Stability

Cited by 2 publications

References 224 publications

A GP1BA Variant in a Czech Family with Monoallelic Bernard-Soulier Syndrome

A GP1BA Variant in a Czech Family with Monoallelic Bernard-Soulier Syndrome

Tetraspanins and vascular functions

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