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Am J Hematol ; 93(3): 430-441, 2018 03.
Artículo en Inglés | MEDLINE | ID: mdl-29247535

RESUMEN

The extracellular matrix (ECM) is a key acellular structure in constant remodeling to provide tissue cohesion and rigidity. Deregulation of the balance between matrix deposition, degradation, and crosslinking results in fibrosis. Bone marrow fibrosis (BMF) is associated with several malignant and nonmalignant pathologies severely affecting blood cell production. BMF results from abnormal deposition of collagen fibers and enhanced lysyl oxidase-mediated ECM crosslinking within the marrow, thereby increasing marrow stiffness. Bone marrow stiffness has been recently recognized as an important regulator of blood cell development, notably by modifying the fate and differentiation process of hematopoietic or mesenchymal stem cells. This review surveys the different components of the ECM and their influence on stem cell development, with a focus on the impact of the ECM composition and stiffness on the megakaryocytic lineage in health and disease. Megakaryocyte maturation and the biogenesis of their progeny, the platelets, are thought to respond to environmental mechanical forces through a number of mechanosensors, including integrins and mechanosensitive ion channels, reviewed here.


Asunto(s)
Plaquetas/citología , Médula Ósea/fisiología , Matriz Extracelular/fisiología , Hematopoyesis/fisiología , Megacariocitos/citología , Animales , Proteínas de la Matriz Extracelular/química , Proteínas de la Matriz Extracelular/fisiología , Glicosaminoglicanos/fisiología , Células Madre Hematopoyéticas/citología , Humanos , Integrinas/fisiología , Canales Iónicos/fisiología , Mecanotransducción Celular , Células Madre Mesenquimatosas/citología , Ratones , Proteínas de Neoplasias/fisiología , Neoplasias/patología , Mielofibrosis Primaria/patología , Proteína-Lisina 6-Oxidasa/fisiología , Trombopoyesis/fisiología
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