Please use this identifier to cite or link to this item:
http://hdl.handle.net/2445/172732
Title: | Phosphotyrosine proteins in platelets from patients with storage pool disease: direct relation between granule defects and defective signal transduction |
Author: | Arderiu i Marquès, Gemma Diaz Ricart, M. Isabel Domènech, Pere Escolar Albaladejo, Ginés Ordinas Bauzá, Antonio Pujol-Moix, Nuria |
Keywords: | Plaquetes sanguínies Metabolisme Patologia Blood platelets Metabolism Pathology |
Issue Date: | 1-Jun-2002 |
Publisher: | Ferrata Storti Foundation |
Abstract: | Background and objectives: storage pool diseases (SPD) are heterogeneous disorders associated with an abnormal presence of intraplatelet granules, which cause mild to moderate bleeding diathesis. We investigated signaling through tyrosine phosphorylation of proteins occurring in platelets with total or partial absence of dense- and alpha-granules in response to activation. Design and methods: we included a patient with severe delta-SPD, a patient with severe alpha-SPD or gray platelet syndrome, and six patients with partial deficiency of dense or a-granules. SPD was confirmed by electron microscopy evaluation of platelet ultrastructure. Platelet function was evaluated by bleeding time determination and conventional aggregometry. Platelet suspensions were activated with collagen and thrombin to analyze changes in tyrosine phosphorylation of proteins by electrophoresis and Western-blotting. Results: bleeding times were prolonged in all the patients included. Aggregation responses were slightly decreased in delta-SPD and normal in the rest of patients. Tyrosine phosphorylation in platelets from patients with partial forms of SPD was equivalent to that observed in control platelets, absent in response to collagen and thrombin activation in delta-SPD, and deficient only to thrombin activation in alpha-SPD. Interpretation and conclusions: tyrosine phosphorylation of proteins in activated platelets is highly dependent on the substances contained in the dense-granules and moderately dependent on those contained in the alpha-granules. A minimum amount of intraplatelet granules ensures signaling through tyrosine phosphorylation of proteins. |
Note: | Reproducció del document publicat a: https://haematologica.org/issue/view/94 |
It is part of: | Haematologica, 2002, vol. 87, num. 6, p. 629-636 |
URI: | http://hdl.handle.net/2445/172732 |
ISSN: | 0390-6078 |
Appears in Collections: | Articles publicats en revistes (Ciències Clíniques) Articles publicats en revistes (Medicina) |
Files in This Item:
File | Description | Size | Format | |
---|---|---|---|---|
545766.pdf | 153.24 kB | Adobe PDF | View/Open |
Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.