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Your search keyword '"De Wilde, Jonathan R.A."' showing total 10 results
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10 results on '"De Wilde, Jonathan R.A."'

2. Osmotic gradient ektacytometry – a novel diagnostic approach for neuroacanthocytosis syndromes

Author

CDL Rode cel, Cancer, CDL Research analisten, Secretariaat Hematologie, Circulatory Health, Poli Van Creveldkliniek Medisch, Child Health, CDL Cluster Speciële Diagnostiek, Hernández, Carolina A., Peikert, Kevin, Qiao, Min, Darras, Alexis, de Wilde, Jonathan R.A., Bos, Jennifer, Leibowitz, Maya, Galea, Ian, Wagner, Christian, Rab, Minke A.E., Walker, Ruth H., Hermann, Andreas, van Beers, Eduard J., van Wijk, Richard, Kaestner, Lars, CDL Rode cel, Cancer, CDL Research analisten, Secretariaat Hematologie, Circulatory Health, Poli Van Creveldkliniek Medisch, Child Health, CDL Cluster Speciële Diagnostiek, Hernández, Carolina A., Peikert, Kevin, Qiao, Min, Darras, Alexis, de Wilde, Jonathan R.A., Bos, Jennifer, Leibowitz, Maya, Galea, Ian, Wagner, Christian, Rab, Minke A.E., Walker, Ruth H., Hermann, Andreas, van Beers, Eduard J., van Wijk, Richard, and Kaestner, Lars

Published
2024

3. Corrigendum to “Activation of pyruvate kinase as therapeutic option for rare hemolytic anemias: Shedding new light on an old enzyme” [Blood Rev. 2023 Sep:61:101103] (Blood Reviews (2023) 61, (S0268960X23000644), (10.1016/j.blre.2023.101103))

Author

CDL Rode cel, Unit Opleiding Aios, Cancer, Haematologie patientenzorg, Poli Van Creveldkliniek Medisch, Child Health, Secretariaat Hematologie, Circulatory Health, CDL Cluster Speciële Diagnostiek, van Dijk, Myrthe J., de Wilde, Jonathan R.A., Bartels, Marije, Kuo, Kevin H.M., Glenthøj, Andreas, Rab, Minke A.E., van Beers, Eduard J., van Wijk, Richard, CDL Rode cel, Unit Opleiding Aios, Cancer, Haematologie patientenzorg, Poli Van Creveldkliniek Medisch, Child Health, Secretariaat Hematologie, Circulatory Health, CDL Cluster Speciële Diagnostiek, van Dijk, Myrthe J., de Wilde, Jonathan R.A., Bartels, Marije, Kuo, Kevin H.M., Glenthøj, Andreas, Rab, Minke A.E., van Beers, Eduard J., and van Wijk, Richard

Published
2024

5. Activation of pyruvate kinase as therapeutic option for rare hemolytic anemias:Shedding new light on an old enzyme

Author

van Dijk, Myrthe J., de Wilde, Jonathan R.A., Bartels, Marije, Kuo, Kevin H.M., Glenthøj, Andreas, Rab, Minke A.E., van Beers, Eduard J., van Wijk, Richard, van Dijk, Myrthe J., de Wilde, Jonathan R.A., Bartels, Marije, Kuo, Kevin H.M., Glenthøj, Andreas, Rab, Minke A.E., van Beers, Eduard J., and van Wijk, Richard

Abstract

Novel developments in therapies for various hereditary hemolytic anemias reflect the pivotal role of pyruvate kinase (PK), a key enzyme of glycolysis, in red blood cell (RBC) health. Without PK catalyzing one of the final steps of the Embden-Meyerhof pathway, there is no net yield of adenosine triphosphate (ATP) during glycolysis, the sole source of energy production required for proper RBC function and survival. In hereditary hemolytic anemias, RBC health is compromised and therefore lifespan is shortened. Although our knowledge on glycolysis in general and PK function in particular is solid, recent advances in genetic, molecular, biochemical, and metabolic aspects of hereditary anemias have improved our understanding of these diseases. These advances provide a rationale for targeting PK as therapeutic option in hereditary hemolytic anemias other than PK deficiency. This review summarizes the knowledge, rationale, (pre)clinical trials, and future advances of PK activators for this important group of rare diseases.

Published
2023

6. An Endogenous Metabolic Modulator Composition Improves Hydration Status and Decreases Adhesion Properties of Red Blood Cells from Patients with Sickle Cell Disease

Author

van Dijk, Myrthe J., primary, Traets, Marissa J.M., additional, van Oirschot, Brigitte A., additional, de Wilde, Jonathan R.A., additional, Bos, Jennifer, additional, van Solinge, Wouter W., additional, Koziel, Margaret J., additional, Wani, Revati, additional, van Beers, Eduard J., additional, Wijk, Richard van, additional, and Rab, Minke A.E., additional

Published
2022
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7. Transfusion Burden in Early Childhood Plays an Important Role in Iron Overload in Diamond-Blackfan Anaemia

Author

Huisman, Elise, de Wilde, Jonathan R.A., van Dooijeweert, B, van Vuren, Annelies, Smiers, Frans, van der Veer, Arian, van Wijk, Richard, van Solinge, W, Nieuwenhuis, Edward E S, van Beers, Eduard J., Bartels, Marije, Huisman, Elise, de Wilde, Jonathan R.A., van Dooijeweert, B, van Vuren, Annelies, Smiers, Frans, van der Veer, Arian, van Wijk, Richard, van Solinge, W, Nieuwenhuis, Edward E S, van Beers, Eduard J., and Bartels, Marije

Abstract

In Diamond-Blackfan anaemia (DBA), iron overload (IO) is common in transfusiondependent patients, yet has also been reported in non-transfusion-dependent patients. We explored the incidence of IO in transfusion-dependent and non-transfusiondependent DBA patients. We observed hepatic IO in 65% of patients analysed with MRI, including three patients that were only treated with transfusions in the past. Whereas overall ferritin levels and liver iron content correlated, ferritin levels did not reflect total body iron adequately. Our data suggest that transfusion burden in the past plays an important role in IO in DBA, and should be taken into account during follow up.

Published
2022

10. The Pyruvate Kinase (PK) Activator AG-946 Improves PK Properties and Red Blood Cell (RBC) Characteristics upon Ex Vivotreatment of RBCs from Patients with Myelodysplastic Syndromes

Author

De Wilde, Jonathan R.A., Ruiter, Titine J.J., van Oirschot, Brigitte A., Jans, Judith J.M., Dang, Lenny, Wind-Rotolo, Megan, Thielen, Noortje, van Solinge, Wouter W., van Rhenen, Anna, van Wijk, Richard, and Rab, Minke A.E.

Abstract

Background:Myelodysplastic syndromes (MDS) consist of a heterogenous group of clonal myeloid malignancies, characterized by dysplasia, ineffective hematopoiesis and cytopenias. MDS patients frequently suffer from anemia, directly affecting quality of life. The limited therapeutic options often do not result in the desired clinical improvement. Interestingly, in MDS the activity of the red blood cell (RBC) enzyme pyruvate kinase (PK), a key regulatory enzyme of glycolysis, may be decreased. The activation of PK via small molecules is hypothesized to be beneficial in a wide range of anemias. Currently, a phase 2a, open-label, proof of concept trial is running on the use of the PK activator AG-946 in low-risk MDS patients (NCT05490446). In light of these developments, we investigated the effect of ex vivotreatment of MDS RBCs with AG-946.

Published
2023
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