Lysosome-related organelles as functional adaptations of the endolysosomal system

C Delevoye, MS Marks, G Raposo - Current opinion in cell biology, 2019 - Elsevier
Unique functions of specialised cells such as those of the immune and haemostasis
systems, skin, blood vessels, lung, and bone require specialised compartments, collectively …

Weibel Palade bodies: unique secretory organelles of endothelial cells that control blood vessel homeostasis

J Naß, J Terglane, V Gerke - Frontiers in Cell and Developmental …, 2021 - frontiersin.org
Vascular endothelial cells produce and release compounds regulating vascular tone, blood
vessel growth and differentiation, plasma composition, coagulation and fibrinolysis, and also …

Coatopathies: genetic disorders of protein coats

EC Dell'Angelica, JS Bonifacino - Annual review of cell and …, 2019 - annualreviews.org
Protein coats are supramolecular complexes that assemble on the cytosolic face of
membranes to promote cargo sorting and transport carrier formation in the endomembrane …

Orchestration of primary hemostasis by platelet and endothelial lysosome-related organelles

E Karampini, R Bierings, J Voorberg - … , thrombosis, and vascular …, 2020 - Am Heart Assoc
Megakaryocyte-derived platelets and endothelial cells store their hemostatic cargo in α-and
δ-granules and Weibel-Palade bodies, respectively. These storage granules belong to the …

Trafficking in blood vessel development

CR Francis, EJ Kushner - Angiogenesis, 2022 - Springer
Blood vessels demonstrate a multitude of complex signaling programs that work in concert
to produce functional vasculature networks during development. A known, but less widely …

New insights into the pathogenesis of Hermansky–Pudlak syndrome

W Li, CJ Hao, ZH Hao, J Ma, QC Wang… - Pigment Cell & …, 2022 - Wiley Online Library
Hermansky–Pudlak syndrome (HPS) is characterized by defects of multiple tissue‐specific
lysosome‐related organelles (LROs), typically manifesting with oculocutaneous albinism or …

GDP/GTP exchange factor MADD drives activation and recruitment of secretory Rab GTPases to Weibel-Palade bodies

M Kat, PE Bürgisser, H Janssen, IM De Cuyper… - Blood …, 2021 - ashpublications.org
Abstract von Willebrand factor (VWF) is an essential hemostatic protein that is synthesized
and secreted by endothelial cells and stored in Weibel-Palade bodies (WPBs). The …

Role of adaptin protein complexes in intracellular trafficking and their impact on diseases

J Shin, A Nile, JW Oh - Bioengineered, 2021 - Taylor & Francis
Adaptin proteins (APs) play a crucial role in intracellular cell trafficking. The 'classical'role of
APs is carried out by AP1‒3, which bind to clathrin, cargo, and accessory proteins …

[HTML][HTML] SYMPHONY consortium: Orchestrating personalized treatment for patients with bleeding disorders

MH Cnossen, I van Moort, SH Reitsma… - Journal of Thrombosis …, 2022 - Elsevier
Background Treatment choices for individual patients with an inborn bleeding disorder are
increasingly challenging due to increasing options and rising costs for society. We have …

A new look at an old body: molecular determinants of Weibel-Palade body composition and VWF exocytosis

S Hordijk, T Carter, R Bierings - Journal of Thrombosis and Haemostasis, 2024 - Elsevier
Endothelial cells, forming a monolayer along blood vessels, intricately regulate vascular
hemostasis, inflammatory responses, and angiogenesis. A key determinant of these …