Phosphotyrosine proteins in platelets from patients with storage pool disease: direct relation between granule defects and defective signal transduction

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...

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Autores: Arderiu i Marquès, Gemma, Diaz Ricart, M. Isabel, Domènech, Pere, Escolar Albaladejo, Ginés, Ordinas Bauzá, Antonio, Pujol-Moix, Nuria
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2002
País:España
Institución:Universidad de Barcelona
Repositorio:Dipòsit Digital de la UB
OAI Identifier:oai:diposit.ub.edu:2445/172732
Acceso en línea:https://hdl.handle.net/2445/172732
Access Level:acceso abierto
Palabra clave:Plaquetes sanguínies
Metabolisme
Patologia
Blood platelets
Metabolism
Pathology
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spelling Phosphotyrosine proteins in platelets from patients with storage pool disease: direct relation between granule defects and defective signal transductionArderiu i Marquès, GemmaDiaz Ricart, M. IsabelDomènech, PereEscolar Albaladejo, GinésOrdinas Bauzá, AntonioPujol-Moix, NuriaPlaquetes sanguíniesMetabolismePatologiaBlood plateletsMetabolismPathologyBackground 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.Ferrata Storti Foundation2002info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://hdl.handle.net/2445/172732Articles publicats en revistes (Medicina)reponame:Dipòsit Digital de la UBinstname:Universidad de BarcelonaInglésReproducció del document publicat a: https://haematologica.org/issue/view/94Haematologica, 2002, vol. 87, num. 6, p. 629-636(c) Ferrata Storti Foundation, 2002info:eu-repo/semantics/openAccessoai:diposit.ub.edu:2445/1727322026-05-27T06:46:51Z
dc.title.none.fl_str_mv Phosphotyrosine proteins in platelets from patients with storage pool disease: direct relation between granule defects and defective signal transduction
title Phosphotyrosine proteins in platelets from patients with storage pool disease: direct relation between granule defects and defective signal transduction
spellingShingle Phosphotyrosine proteins in platelets from patients with storage pool disease: direct relation between granule defects and defective signal transduction
Arderiu i Marquès, Gemma
Plaquetes sanguínies
Metabolisme
Patologia
Blood platelets
Metabolism
Pathology
title_short Phosphotyrosine proteins in platelets from patients with storage pool disease: direct relation between granule defects and defective signal transduction
title_full Phosphotyrosine proteins in platelets from patients with storage pool disease: direct relation between granule defects and defective signal transduction
title_fullStr Phosphotyrosine proteins in platelets from patients with storage pool disease: direct relation between granule defects and defective signal transduction
title_full_unstemmed Phosphotyrosine proteins in platelets from patients with storage pool disease: direct relation between granule defects and defective signal transduction
title_sort Phosphotyrosine proteins in platelets from patients with storage pool disease: direct relation between granule defects and defective signal transduction
dc.creator.none.fl_str_mv Arderiu i Marquès, Gemma
Diaz Ricart, M. Isabel
Domènech, Pere
Escolar Albaladejo, Ginés
Ordinas Bauzá, Antonio
Pujol-Moix, Nuria
author Arderiu i Marquès, Gemma
author_facet Arderiu i Marquès, Gemma
Diaz Ricart, M. Isabel
Domènech, Pere
Escolar Albaladejo, Ginés
Ordinas Bauzá, Antonio
Pujol-Moix, Nuria
author_role author
author2 Diaz Ricart, M. Isabel
Domènech, Pere
Escolar Albaladejo, Ginés
Ordinas Bauzá, Antonio
Pujol-Moix, Nuria
author2_role author
author
author
author
author
dc.subject.none.fl_str_mv Plaquetes sanguínies
Metabolisme
Patologia
Blood platelets
Metabolism
Pathology
topic Plaquetes sanguínies
Metabolisme
Patologia
Blood platelets
Metabolism
Pathology
description 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.
publishDate 2002
dc.date.none.fl_str_mv 2002
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://hdl.handle.net/2445/172732
url https://hdl.handle.net/2445/172732
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv Reproducció del document publicat a: https://haematologica.org/issue/view/94
Haematologica, 2002, vol. 87, num. 6, p. 629-636
dc.rights.none.fl_str_mv (c) Ferrata Storti Foundation, 2002
info:eu-repo/semantics/openAccess
rights_invalid_str_mv (c) Ferrata Storti Foundation, 2002
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Ferrata Storti Foundation
publisher.none.fl_str_mv Ferrata Storti Foundation
dc.source.none.fl_str_mv Articles publicats en revistes (Medicina)
reponame:Dipòsit Digital de la UB
instname:Universidad de Barcelona
instname_str Universidad de Barcelona
reponame_str Dipòsit Digital de la UB
collection Dipòsit Digital de la UB
repository.name.fl_str_mv
repository.mail.fl_str_mv
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