[Murine models of platelet diseases].

Abstract

Platelet-related diseases correspond to functional defects or abnormal production (thrombopoiesis) of hereditary and immunological origins. Recent progress in the manipulation of the mouse genome (transgenesis, gene inactivation or insertion) has resulted in the generation of numerous strains exhibiting defective platelet function or production. Some strains reproduce known hereditary diseases affecting haemostasis (Glanzmann thrombasthenia, Bernard-Soulier syndrome (BSS) or thrombopoiesis (Wiscott-Aldrich or May-Hegglin syndrome). More often the mutated strains have no human equivalent and represent useful models to study: (i) the role of adhesive or signalling receptors or of signalling proteins in platelet-dependent haemostasis and thrombosis or; (ii) to study the poorly characterized mechanisms of thrombopoiesis, which implicate transcription factors (GATA, Fli1), growth factors and receptors (TPO, cMPL), and cytoskeletal or contractile proteins (tubulin, myosin). Additional mouse strains result from the selection of spontaneous mutants many of which affect intracellular platelet granules, representing models of storage pool diseases (SPD) such as the Gray platelet syndrome (alphaSPD) or Hermansky-Pudlack syndrome (deltaSPD). More recently, a systematic chemical mutagenesis approach has also identified genes involved in thrombopoiesis and platelet survival. Finally, mouse models of auto- or allo-immune thrombocytopenia have been developed to study the mechanisms of platelet destruction or removal.

• Authors: F Lanza
• Journal: Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguine (Transfus Clin Biol) Vol. 14 Issue 1 Pg. 35-40 (May 2007) ISSN: 1246-7820 [Print] France
• Vernacular Title: Modèles murins de pathologies plaquettaires.
• PMID: 17500022 (Publication Type: English Abstract, Journal Article, Review)

Topics

• Animals
• Blood Platelet Disorders (classification, genetics)
• Disease Models, Animal
• Genome
• Hemostasis
• Mice
• Mutation
• Thrombocytopenia (classification, genetics)