Nbeal2 Deficiency Increases Organ Damage but Does Not Affect Host Defense During Gram-Negative Pneumonia-Derived Sepsis.
Claushuis, Theodora A M; de Stoppelaar, Sacha F; de Vos, Alex F; Grootemaat, Anita E; van der Wel, Nicole N; Roelofs, Joris J T H; Ware, Jerry; Van't Veer, Cornelis; van der Poll, Tom.
Arterioscler Thromb Vasc Biol
; 38(8): 1772-1784, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29930006
NBEAL2 mutations and bleeding in patients with gray platelet syndrome.
Platelet α-granules modulate the inflammatory response under systemic lipopolysaccharide injection in mice.
Correlation between platelet phenotype and NBEAL2 genotype in patients with congenital thrombocytopenia and α-granule deficiency.
Idiopathic Purpura With Gray Platelets: an Acquired Form of Gray Platelet Syndrome.
Megakaryocytic emperipolesis and platelet function abnormalities in five patients with gray platelet syndrome.
Gray platelet syndrome: proinflammatory megakaryocytes and α-granule loss cause myelofibrosis and confer metastasis resistance in mice.
A novel nonsense NBEAL2 gene mutation causing severe bleeding in a patient with gray platelet syndrome.
VPS33B regulates protein sorting into and maturation of α-granule progenitor organelles in mouse megakaryocytes.
Platelet secretion paves the way.
α-granule biogenesis: from disease to discovery.