Platelet membrane glycoproteins implicated in ristocetin-induced aggregation. Studies of the proteins on platelets from patients with Bernard-Soulier syndrome and von Willebrand's disease.

Jenkins, C. S. P.; Phillips, David R.; Clemetson, Kenneth J.; Meyer, Dominique; Larrieu, M J; Lüscher, E. F.

doi:10.1172/jci108251

Cited by 208 publications

(67 citation statements)

References 32 publications

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“…In view of the apparent contradiction between our initial observations and those of Jenkins et al (14), the increased complexity of the human platelet membrane GP composition as revealed by the introduction of improved SDS-PAGE procedures, and the lack of agreement in the reports on the analysis of B-S platelets by crossed immunoelectrophoresis, we now describe further studies performed on the platelets of four B-S patients. Analysis ofthe GP and proteins of 125I-labeled platelets of each patient by refined single and two-dimensional SDS-PAGE procedures has established that B-S platelets possess a specific and characteristic membrane GP lesion.…”

Section: Introductioncontrasting

confidence: 60%

“…This abnormality was confirmed by the direct analysis of unfractionated B-S platelets by SDS-PAGE (3). In contrast, Jenkins et al (14) reported other findings after their studies on the platelets of a different B-S patient. These authors detected a diffuse PAS band in the GP I position after SDS-PAGE and were unable to detect PAS-staining in the GP II and III positions.…”

Section: Introductionmentioning

confidence: 88%

“…In particular, three large polypeptides with apparent molecular weights of 250,000, 230,000, and 200,000 were normally detected. These polypeptides, which have been shown to be highly sensitive to degradation by an endogenous platelet calcium-sensitive protease (33), have been reported to be modified in B-S platelets (14). As shown in Table I, the total protein content of B-S platelets was increased relative to nonnal human platelets.…”

Section: Neuraminidase Digestionmentioning

confidence: 96%

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Analysis of the glycoprotein and protein composition of Bernard-Soulier platelets by single and two-dimensional sodium dodecyl sulfate-polyacrylamide gel electrophoresis.

Nurden¹,

Dupuis²,

Kunicki³

et al. 1981

J. Clin. Invest.

147

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A B S T R A C T Previous reports have described conflicting results concerning the glycoprotein (GP) and protein composition of Bernard-Soulier platelets. In view of this controversy we have analyzed the platelets of four Bernard-Soulier patients using improved single and two-dimensional sodium dodecyl sulfate (SDS)-polyacrylamide gel electrophoresis procedures. An absence of staining for carbohydrate of membrane GP lb was characteristic for the platelets of each patient. Major periodate-Schiff staining bands corresponding to membrane GP lIb, Illa, and IlIb were clearly detected and their presence was confirmed by two-dimensional SDS-polyacrylamide gel electrophoresis. The protein content of the Bernard-Soulier platelets was increased two-to fourfold. However, analysis of their protein composition using 7-12% acrylamide gradient gels showed normal polypeptide profiles. Lactoperoxidase-catalyzed 125I-labeling of the Bernard-Soulier platelet surface proteins was followed by SDS-polyacrylamide gel electrophoresis and autoradiography. No labeling in the Ib position was detected whereas the other major membrane GP, including Ia and Ila, were normally located. In contrast, GP lb was clearly detected by periodate-Schiff staining and autoradiography when normal human platelets that had been exhaustively treated with neuraminidase before the lactoperoxidase-catalyzed iodination were analysed. No abnormalities were detected in the GP patterns of membranes isolated from the patients' erythrocytes. Only a severe molecular abnormality or possible deletion of GP lb could account for this major platelet lesion in the Bernard-Soulier syndrome.

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Section: Introductioncontrasting

confidence: 60%

Section: Introductionmentioning

confidence: 88%

Section: Neuraminidase Digestionmentioning

confidence: 96%

See 1 more Smart Citation

Analysis of the glycoprotein and protein composition of Bernard-Soulier platelets by single and two-dimensional sodium dodecyl sulfate-polyacrylamide gel electrophoresis.

Nurden¹,

Dupuis²,

Kunicki³

et al. 1981

J. Clin. Invest.

147

View full text Add to dashboard Cite

show abstract

“…Two-dimensional gel electrophoresis (2D-GE) (30), which separates proteins by both size and charge, is the basis of many current proteomic techniques but has been used for many years to study platelet biology (31,32). Initial studies using reducing and nonreducing gels resulted in the naming of many of the platelet glycoproteins and the identification of protein defects in patients with inherited bleeding disorders (2).…”

Section: Proteomicsmentioning

confidence: 99%

Platelet genomics and proteomics in human health and disease

Macaulay

Carr²,

Gusnanto³

et al. 2005

Journal of Clinical Investigation

150

100

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Proteomic and genomic technologies provide powerful tools for characterizing the multitude of events that occur in the anucleate platelet. These technologies are beginning to define the complete platelet transcriptome and proteome as well as the protein-protein interactions critical for platelet function. The integration of these results provides the opportunity to identify those proteins involved in discrete facets of platelet function. Here we summarize the findings of platelet proteome and transcriptome studies and their application to diseases of platelet function.

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“…Platelet glycoprotein GPIb is reduced in CDG-IIf and is reduced or absent in Bernard-Soulier syndrome. 3,29,30 In contrast to this, the patient's platelets showed normal GPIb expression ( Figure 4C), further excluding the presence of one of the above-mentioned diseases.…”

Section: No Functional Mutations In the Gene Coding For The Cmp-sialimentioning

confidence: 77%

A Novel Type of Macrothrombocytopenia Associated with a Defect in α2,3-Sialylation

Jones

Denecke

Sträter

et al. 2011

The American Journal of Pathology

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We describe a novel type of human thrombocytopenia characterized by the appearance of giant platelets and variable neutropenia. Searching for the molecular defect, we found that neutrophils had strongly reduced sialyl-Lewis X and increased Lewis X surface expression, pointing to a deficiency in sialylation. We show that the glycosylation defect is restricted to ␣2,3-sialylation and can be detected in platelets, neutrophils, and monocytes. Platelets exhibited a distorted structure of the open canalicular system, indicating defective platelet generation. Importantly, patient platelets, but not normal platelets, bound to the asialoglycoprotein receptor (ASGP-R), a liver cellsurface protein that removes desialylated thrombocytes from the circulation in mice. Taken together, this is the first type of human thrombocytopenia in which a specific defect of ␣2,3-sialylation and an induction of platelet binding to the liver ASGP-R could be detected.

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Platelet membrane glycoproteins implicated in ristocetin-induced aggregation. Studies of the proteins on platelets from patients with Bernard-Soulier syndrome and von Willebrand's disease.

Cited by 208 publications

References 32 publications

Analysis of the glycoprotein and protein composition of Bernard-Soulier platelets by single and two-dimensional sodium dodecyl sulfate-polyacrylamide gel electrophoresis.

Analysis of the glycoprotein and protein composition of Bernard-Soulier platelets by single and two-dimensional sodium dodecyl sulfate-polyacrylamide gel electrophoresis.

Platelet genomics and proteomics in human health and disease

A Novel Type of Macrothrombocytopenia Associated with a Defect in α2,3-Sialylation

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