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  1. Article: Inside Out Integrin Activation Mediated by PIEZO1 Signaling in Erythroblasts.

    Aglialoro, Francesca / Hofsink, Naomi / Hofman, Menno / Brandhorst, Nicole / van den Akker, Emile

    Frontiers in physiology

    2020  Volume 11, Page(s) 958

    Abstract: The non-selective mechanosensitive ion channel PIEZO1 controls erythrocyte volume homeostasis. Different missense gain-of-function mutations ... ...

    Abstract The non-selective mechanosensitive ion channel PIEZO1 controls erythrocyte volume homeostasis. Different missense gain-of-function mutations in
    Language English
    Publishing date 2020-07-31
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2564217-0
    ISSN 1664-042X
    ISSN 1664-042X
    DOI 10.3389/fphys.2020.00958
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Mutations in Neurobeachin-like 2 do not impact Weibel-Palade body biogenesis and von Willebrand factor secretion in gray platelet syndrome Endothelial Colony Forming Cells.

    Kat, Marije / van Moort, Iris / Bürgisser, Petra E / Kuijpers, Taco W / Hofman, Menno / Favier, Marie / Favier, Rémi / Margadant, Coert / Voorberg, Jan / Bierings, Ruben

    Research and practice in thrombosis and haemostasis

    2023  Volume 7, Issue 2, Page(s) 100086

    Abstract: Background: Patients with gray platelet syndrome (GPS) and Neurobeachin-like 2 (NBEAL2) deficiency produce platelets lacking alpha-granules (AGs) and present with lifelong bleeding symptoms. AGs are lysosome-related organelles and store the hemostatic ... ...

    Abstract Background: Patients with gray platelet syndrome (GPS) and Neurobeachin-like 2 (NBEAL2) deficiency produce platelets lacking alpha-granules (AGs) and present with lifelong bleeding symptoms. AGs are lysosome-related organelles and store the hemostatic protein von Willebrand factor (VWF) and the transmembrane protein P-selectin. Weibel-Palade bodies (WPBs) are lysosome-related organelles of endothelial cells and also store VWF and P-selectin. In megakaryocytes, NBEAL2 links P-selectin on AGs to the SNARE protein SEC22B on the endoplasmic reticulum, thereby preventing premature release of cargo from AG precursors. In endothelial cells, SEC22B drives VWF trafficking from the endoplasmic reticulum to Golgi and promotes the formation of elongated WPBs, but it is unclear whether this requires NBEAL2.
    Objectives: To investigate a potential role for NBEAL2 in WPB biogenesis and VWF secretion using NBEAL2-deficient endothelial cells.
    Methods: The interaction of SEC22B with NBEAL2 in endothelial cells was investigated by interatomic mass spectrometry and pull-down analysis. Endothelial colony forming cells were isolated from healthy controls and 3 unrelated patients with GPS and mutations in
    Results: We showed that SEC22B binds to NBEAL2 in ECs. Endothelial colony forming cells derived from a patient with GPS are deficient in NBEAL2 but reveal normal formation and maturation of WPBs and normal WPB cargo recruitment. Neither basal nor histamine-induced VWF secretion is altered in the absence of NBEAL2.
    Conclusions: Although NBEAL2 deficiency causes the absence of AGs in patients with GPS, it does not impact WPB functionality in ECs. Our data highlight the differences in the regulatory mechanisms between these 2 hemostatic storage compartments.
    Language English
    Publishing date 2023-02-14
    Publishing country United States
    Document type Journal Article
    ISSN 2475-0379
    ISSN (online) 2475-0379
    DOI 10.1016/j.rpth.2023.100086
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Alternative trafficking of Weibel-Palade body proteins in CRISPR/Cas9-engineered von Willebrand factor-deficient blood outgrowth endothelial cells.

    Schillemans, Maaike / Kat, Marije / Westeneng, Jurjen / Gangaev, Anastasia / Hofman, Menno / Nota, Benjamin / van Alphen, Floris P J / de Boer, Martin / van den Biggelaar, Maartje / Margadant, Coert / Voorberg, Jan / Bierings, Ruben

    Research and practice in thrombosis and haemostasis

    2019  Volume 3, Issue 4, Page(s) 718–732

    Abstract: Background: Synthesis of the hemostatic protein von Willebrand factor (VWF) drives formation of endothelial storage organelles called Weibel-Palade bodies (WPBs). In the absence of VWF, angiogenic and inflammatory mediators that are costored in WPBs are ...

    Abstract Background: Synthesis of the hemostatic protein von Willebrand factor (VWF) drives formation of endothelial storage organelles called Weibel-Palade bodies (WPBs). In the absence of VWF, angiogenic and inflammatory mediators that are costored in WPBs are subject to alternative trafficking routes. In patients with von Willebrand disease (VWD), partial or complete absence of VWF/WPBs may lead to additional bleeding complications, such as angiodysplasia. Studies addressing the role of VWF using VWD patient-derived blood outgrowth endothelial cells (BOECs) have reported conflicting results due to the intrinsic heterogeneity of patient-derived BOECs.
    Objective: To generate a VWF-deficient endothelial cell model using clustered regularly interspaced short palindromic repeats (CRISPR) genome engineering of blood outgrowth endothelial cells.
    Methods: We used CRISPR/CRISPR-associated protein 9 editing in single-donor cord blood-derived BOECs (cbBOECs) to generate clonal
    Results: Two
    Conclusions: CRISPR editing of
    Language English
    Publishing date 2019-08-01
    Publishing country United States
    Document type Journal Article
    ISSN 2475-0379
    ISSN (online) 2475-0379
    DOI 10.1002/rth2.12242
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Defective AP-3-dependent VAMP8 trafficking impairs Weibel-Palade body exocytosis in Hermansky-Pudlak Syndrome type 2 blood outgrowth endothelial cells.

    Karampini, Ellie / Schillemans, Maaike / Hofman, Menno / van Alphen, Floris / de Boer, Martin / Kuijpers, Taco W / van den Biggelaar, Maartje / Voorberg, Jan / Bierings, Ruben

    Haematologica

    2019  Volume 104, Issue 10, Page(s) 2091–2099

    Abstract: Weibel-Palade bodies are endothelial secretory organelles that contain von Willebrand factor, P-selectin and CD63. Release of von Willebrand factor from Weibel-Palade bodies is crucial for platelet adhesion during primary hemostasis. Endosomal ... ...

    Abstract Weibel-Palade bodies are endothelial secretory organelles that contain von Willebrand factor, P-selectin and CD63. Release of von Willebrand factor from Weibel-Palade bodies is crucial for platelet adhesion during primary hemostasis. Endosomal trafficking of proteins like CD63 to Weibel-Palade bodies during maturation is dependent on the adaptor protein complex 3 complex. Mutations in the
    MeSH term(s) Adaptor Protein Complex 3/genetics ; Adaptor Protein Complex 3/metabolism ; Adaptor Protein Complex beta Subunits/genetics ; Adaptor Protein Complex beta Subunits/metabolism ; Calcium Signaling ; Cells, Cultured ; Endothelial Cells/metabolism ; Endothelial Cells/pathology ; Exocytosis ; Hermanski-Pudlak Syndrome/genetics ; Hermanski-Pudlak Syndrome/metabolism ; Hermanski-Pudlak Syndrome/pathology ; Humans ; Mutation ; Protein Transport ; R-SNARE Proteins/genetics ; R-SNARE Proteins/metabolism ; Weibel-Palade Bodies/genetics ; Weibel-Palade Bodies/metabolism ; Weibel-Palade Bodies/pathology
    Chemical Substances AP3B1 protein, human ; Adaptor Protein Complex 3 ; Adaptor Protein Complex beta Subunits ; R-SNARE Proteins ; VAMP8 protein, human
    Language English
    Publishing date 2019-01-10
    Publishing country Italy
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2333-4
    ISSN 1592-8721 ; 0017-6567 ; 0390-6078
    ISSN (online) 1592-8721
    ISSN 0017-6567 ; 0390-6078
    DOI 10.3324/haematol.2018.207787
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Uh III Zs.76: Show issues Location:
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    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

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  5. Article ; Online: Establishment of outgrowth endothelial cells from peripheral blood.

    Martin-Ramirez, Javier / Hofman, Menno / van den Biggelaar, Maartje / Hebbel, Robert P / Voorberg, Jan

    Nature protocols

    2012  Volume 7, Issue 9, Page(s) 1709–1715

    Abstract: Blood outgrowth endothelial cells (BOECs) are important tools when investigating diagnostic and therapeutic approaches for vascular disease. In this protocol, mononuclear cells are isolated from peripheral blood and plated on type I collagen at ∼135,000 ... ...

    Abstract Blood outgrowth endothelial cells (BOECs) are important tools when investigating diagnostic and therapeutic approaches for vascular disease. In this protocol, mononuclear cells are isolated from peripheral blood and plated on type I collagen at ∼135,000 cells per cm(2) in endothelial cell differentiation medium. On average, 0.34 colonies of endothelial cells per milliliter of blood can be obtained. Colonies of endothelial cells become visible after 14-28 d. Upon confluence, these rapidly expanding colonies can be passaged and have been shown to propagate up to 10(18)-fold. Isolated BOECs are phenotypically similar to vascular endothelial cells, as revealed by their cobblestone morphology, the presence of endothelial cell-specific Weibel-Palade bodies and the expression of endothelial cell markers such as VE-cadherin. The protocol presented here also provides a particularly useful tool for the ex vivo assessment of endothelial cell function from patients with different vascular abnormalities.
    MeSH term(s) Antigens, CD/metabolism ; Blood Cells/cytology ; Cadherins/metabolism ; Cell Culture Techniques/methods ; Cell Differentiation/physiology ; Endothelial Cells/cytology ; Humans ; Phenotype ; Vascular Diseases/diagnosis
    Chemical Substances Antigens, CD ; Cadherins ; cadherin 5
    Language English
    Publishing date 2012-08-23
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 2244966-8
    ISSN 1750-2799 ; 1754-2189
    ISSN (online) 1750-2799
    ISSN 1754-2189
    DOI 10.1038/nprot.2012.093
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  6. Article ; Online: Weibel-Palade Body Localized Syntaxin-3 Modulates Von Willebrand Factor Secretion From Endothelial Cells.

    Schillemans, Maaike / Karampini, Ellie / van den Eshof, Bart L / Gangaev, Anastasia / Hofman, Menno / van Breevoort, Dorothee / Meems, Henriët / Janssen, Hans / Mulder, Aat A / Jost, Carolina R / Escher, Johanna C / Adam, Rüdiger / Carter, Tom / Koster, Abraham J / van den Biggelaar, Maartje / Voorberg, Jan / Bierings, Ruben

    Arteriosclerosis, thrombosis, and vascular biology

    2018  Volume 38, Issue 7, Page(s) 1549–1561

    Abstract: Objective: Endothelial cells store VWF (von Willebrand factor) in rod-shaped secretory organelles, called Weibel-Palade bodies (WPBs). WPB exocytosis is coordinated by a complex network of Rab GTPases, Rab effectors, and SNARE (soluble NSF attachment ... ...

    Abstract Objective: Endothelial cells store VWF (von Willebrand factor) in rod-shaped secretory organelles, called Weibel-Palade bodies (WPBs). WPB exocytosis is coordinated by a complex network of Rab GTPases, Rab effectors, and SNARE (soluble NSF attachment protein receptor) proteins. We have previously identified STXBP1 as the link between the Rab27A-Slp4-a complex on WPBs and the SNARE proteins syntaxin-2 and -3. In this study, we investigate the function of syntaxin-3 in VWF secretion.
    Approach and results: In human umbilical vein endothelial cells and in blood outgrowth endothelial cells (BOECs) from healthy controls, endogenous syntaxin-3 immunolocalized to WPBs. A detailed analysis of BOECs isolated from a patient with variant microvillus inclusion disease, carrying a homozygous mutation in
    Conclusions: Our data reveal syntaxin-3 as a novel WPB-associated SNARE protein that controls WPB exocytosis.
    MeSH term(s) Calcium/metabolism ; Cells, Cultured ; Cyclic AMP/metabolism ; Endothelial Cells/metabolism ; Endothelial Cells/ultrastructure ; Exocytosis ; Human Umbilical Vein Endothelial Cells/metabolism ; Humans ; Malabsorption Syndromes/diagnosis ; Malabsorption Syndromes/genetics ; Malabsorption Syndromes/metabolism ; Microvilli/genetics ; Microvilli/metabolism ; Microvilli/pathology ; Mucolipidoses/diagnosis ; Mucolipidoses/genetics ; Mucolipidoses/metabolism ; Mutation ; Qa-SNARE Proteins/genetics ; Qa-SNARE Proteins/metabolism ; R-SNARE Proteins/metabolism ; Secretory Pathway ; Signal Transduction ; Weibel-Palade Bodies/metabolism ; Weibel-Palade Bodies/ultrastructure ; von Willebrand Factor/metabolism
    Chemical Substances Qa-SNARE Proteins ; R-SNARE Proteins ; VAMP8 protein, human ; von Willebrand Factor ; Cyclic AMP (E0399OZS9N) ; Calcium (SY7Q814VUP)
    Language English
    Publishing date 2018-06-07
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1221433-4
    ISSN 1524-4636 ; 1079-5642
    ISSN (online) 1524-4636
    ISSN 1079-5642
    DOI 10.1161/ATVBAHA.117.310701
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1705: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
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  7. Article ; Online: Individual with subclinical atherosclerosis have impaired proliferation of blood outgrowth endothelial cells, which can be restored by statin therapy.

    Martin-Ramirez, Javier / Kok, Maayke G M / Hofman, Menno / Bierings, Ruben / Creemers, Esther E / Meijers, Joost C M / Voorberg, Jan / Pinto-Sietsma, Sara-Joan

    PloS one

    2014  Volume 9, Issue 6, Page(s) e99890

    Abstract: Background: To study the regenerative capacity of the endothelium in patients with coronary artery disease (CAD), we cultured blood outgrowth endothelial cells (BOECs) of patients with premature CAD and their first degree relatives (FDR). Additionally ... ...

    Abstract Background: To study the regenerative capacity of the endothelium in patients with coronary artery disease (CAD), we cultured blood outgrowth endothelial cells (BOECs) of patients with premature CAD and their first degree relatives (FDR). Additionally we evaluated the influence of statin treatment on circulating BOEC precursors in subjects with subclinical atherosclerosis.
    Methods and results: Patients with premature CAD (men <51 yr, women <56 yr) and their FDRs were included. Based on coronary calcification (CAC) scores FDRs were divided in a group of healthy subjects (CAC = 0) and subjects with subclinical atherosclerosis (CAC>0). We did not observe differences in the number of BOEC colonies and proliferation between premature CAD patients and FDRs. FDRs with subclinical atherosclerosis had lower colony numbers compared with healthy FDRs, however this was not statistically significant, and BOEC proliferation was significantly impaired (OR = 0.45, 95% CI 0.21-0.96). Unexpectedly, the number of BOEC colonies and BOEC proliferation were similar for premature CAD patients and healthy FDRs. Since a considerable number of premature CAD patients used statins, we studied the number of BOEC precursors as well as their proliferative capacity in ten individuals with subclinical atherosclerosis, before and after statin therapy. Interestingly, FDRs with subclinical atherosclerosis showed a significant increase in the number of BOEC colonies after statin therapy.
    Conclusion: BOEC proliferation of subjects with subclinical atherosclerosis is impaired compared with healthy controls. In these subjects, statin therapy significantly increased the number of circulating BOEC precursors as well as their proliferative capacity, revealing a beneficial effect of statins on endothelial regeneration.
    MeSH term(s) Adult ; Aged ; Atherosclerosis/drug therapy ; Atherosclerosis/metabolism ; Atherosclerosis/pathology ; Cell Proliferation/drug effects ; Endothelial Cells/metabolism ; Endothelial Cells/pathology ; Female ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors/administration & dosage ; Male ; Middle Aged ; Stem Cells/metabolism ; Stem Cells/pathology
    Chemical Substances Hydroxymethylglutaryl-CoA Reductase Inhibitors
    Language English
    Publishing date 2014-06-23
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ISSN 1932-6203
    ISSN (online) 1932-6203
    DOI 10.1371/journal.pone.0099890
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