Platelets as key factors in inflammation: focus on CD40L/CD40

F Cognasse, AC Duchez, E Audoux… - Frontiers in …, 2022 - frontiersin.org
Platelets are anucleate cytoplasmic fragments derived from the fragmentation of medullary
megakaryocytes. Activated platelets adhere to the damaged endothelium by means of …

A review of inherited platelet disorders with guidelines for their management on behalf of the UKHCDO

PHB Bolton‐Maggs, EA Chalmers… - British journal of …, 2006 - Wiley Online Library
The inherited platelet disorders are an uncommon cause of symptomatic bleeding. They
may be difficult to diagnose (and are likely to be under‐diagnosed) and pose problems in …

Loss of the Arp2/3 complex component ARPC1B causes platelet abnormalities and predisposes to inflammatory disease

WHA Kahr, FG Pluthero, A Elkadri, N Warner… - Nature …, 2017 - nature.com
Abstract Human actin-related protein 2/3 complex (Arp2/3), required for actin filament
branching, has two ARPC1 component isoforms, with ARPC1B prominently expressed in …

Polyphosphate modulates blood coagulation and fibrinolysis

SA Smith, NJ Mutch, D Baskar… - Proceedings of the …, 2006 - National Acad Sciences
Inorganic polyphosphate is an abundant component of acidocalcisomes of bacteria and
unicellular eukaryotes. Human platelet dense granules strongly resemble acidocalcisomes …

Disorders of lysosome-related organelle biogenesis: clinical and molecular genetics

M Huizing, A Helip-Wooley, W Westbroek… - … Rev. Genomics Hum …, 2008 - annualreviews.org
Lysosome-related organelles (LROs) are a heterogeneous group of vesicles that share
various features with lysosomes, but are distinct in function, morphology, and composition …

Platelet secretion is kinetically heterogeneous in an agonist-responsive manner

D Jonnalagadda, LT Izu… - Blood, The Journal of the …, 2012 - ashpublications.org
Platelets release numerous bioactive molecules stored in their granules enabling them to
exert a wide range of effects on the vascular microenvironment. Are these granule cargo …

NBEAL2 is mutated in gray platelet syndrome and is required for biogenesis of platelet α-granules

M Gunay-Aygun, TC Falik-Zaccai, T Vilboux… - Nature …, 2011 - nature.com
Gray platelet syndrome (GPS) is an autosomal recessive bleeding disorder that is
characterized by large platelets that lack α-granules. Here we show that mutations in …

Hermansky–Pudlak syndrome: mutation update

M Huizing, MCV Malicdan, JA Wang… - Human …, 2020 - Wiley Online Library
Hermansky–Pudlak syndrome (HPS) is a group of 10 autosomal recessive multisystem
disorders, each defined by the deficiency of a specific gene. HPS‐associated genes encode …

Congenital disorders associated with platelet dysfunctions

P Nurden, AT Nurden - Thrombosis and haemostasis, 2008 - thieme-connect.com
Genetic defects of the megakaryocyte lineage give rise to bleeding syndromes of varying
severity. Blood platelets are unable to fulfill their hemostatic function of preventing blood loss …

The gray platelet syndrome: clinical spectrum of the disease

AT Nurden, P Nurden - Blood reviews, 2007 - Elsevier
The gray platelet syndrome (GPS) is a rare inherited disorder of the megakaryocyte (MK)
lineage. Thrombocytopenia and enlarged platelets are associated with a specific absence of …