Search Results - "Mulder, Aat"
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Human-iPSC-Derived Cardiac Stromal Cells Enhance Maturation in 3D Cardiac Microtissues and Reveal Non-cardiomyocyte Contributions to Heart Disease
Published in Cell stem cell (04-06-2020)“…Cardiomyocytes (CMs) from human induced pluripotent stem cells (hiPSCs) are functionally immature, but this is improved by incorporation into engineered…”
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2
Postmortem MRI and histology demonstrate differential iron accumulation and cortical myelin organization in early- and late-onset Alzheimer's disease
Published in Neurobiology of aging (01-02-2018)“…Previous MRI studies reported cortical iron accumulation in early-onset (EOAD) compared to late-onset (LOAD) Alzheimer disease patients. However, the pattern…”
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Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy Family Members With a Pathogenic NOTCH3 Variant Can Have a Normal Brain Magnetic Resonance Imaging and Skin Biopsy Beyond Age 50 Years
Published in Stroke (1970) (01-06-2022)“…To determine whether extremely mild small vessel disease (SVD) phenotypes can occur in variant carriers from Cerebral Autosomal Dominant Arteriopathy with…”
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Weibel-Palade Body Localized Syntaxin-3 Modulates Von Willebrand Factor Secretion From Endothelial Cells
Published in Arteriosclerosis, thrombosis, and vascular biology (01-07-2018)“…OBJECTIVE—Endothelial cells store VWF (von Willebrand factor) in rod-shaped secretory organelles, called Weibel-Palade bodies (WPBs). WPB exocytosis is…”
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5
Bacteriophage ISP eliminates Staphylococcus aureus in planktonic phase, but not in the various stages of the biofilm cycle
Published in Scientific reports (22-06-2024)“…Metal-implant associated bacterial infections are a major clinical problem due to antibiotic treatment failure. As an alternative, we determined the effects of…”
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Syntaxin 5 determines Weibel-Palade body size and Von Willebrand factor secretion by controlling Golgi architecture
Published in Haematologica (Roma) (01-08-2022)“…Von Willebrand factor (VWF) is a multimeric hemostatic protein primarily synthesized in endothelial cells (ECs). VWF is stored in endothelial storage…”
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7
NOTCH3 variant position is associated with NOTCH3 aggregation load in CADASIL vasculature
Published in Neuropathology and applied neurobiology (01-02-2022)“…Aims CADASIL, the most prevalent hereditary cerebral small vessel disease, is caused by cysteine‐altering NOTCH3 variants (NOTCH3cys) leading to vascular…”
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8
Interplay between calcium and sarcomeres directs cardiomyocyte maturation during regeneration
Published in Science (American Association for the Advancement of Science) (19-05-2023)“…Zebrafish hearts can regenerate by replacing damaged tissue with new cardiomyocytes. Although the steps leading up to the proliferation of surviving…”
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Smad6 determines BMP-regulated invasive behaviour of breast cancer cells in a zebrafish xenograft model
Published in Scientific reports (26-04-2016)“…The transforming growth factor-β (TGF-β) family is known to play critical roles in cancer progression. While the dual role of TGF-β is well described, the…”
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10
Tumor Pigmentation Does Not Affect Light-Activated Belzupacap Sarotalocan Treatment but Influences Macrophage Polarization in a Murine Melanoma Model
Published in Investigative ophthalmology & visual science (25-01-2024)“…Pigmentation in uveal melanoma is associated with increased malignancy and is known as a barrier for photodynamic therapy. We investigated the role of…”
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Loss of CRB2 in Müller glial cells modifies a CRB1-associated retinitis pigmentosa phenotype into a Leber congenital amaurosis phenotype
Published in Human molecular genetics (01-01-2019)“…Abstract Variations in the human Crumbs homolog-1 (CRB1) gene lead to an array of retinal dystrophies including early onset of retinitis pigmentosa (RP) and…”
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CRB2 Loss in Rod Photoreceptors Is Associated with Progressive Loss of Retinal Contrast Sensitivity
Published in International journal of molecular sciences (21-08-2019)“…Variations in the Crumbs homolog-1 ( ) gene are associated with a wide variety of autosomal recessive retinal dystrophies, including early onset retinitis…”
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Red blood cells as oxygen carrier during normothermic machine perfusion of kidney grafts: Friend or foe?
Published in American journal of transplantation (01-07-2024)“…Renal ex vivo normothermic machine perfusion (NMP) is under development as an assessment tool for high-risk kidney grafts and as a means of achieving more…”
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In vitro modelling of alveolar repair at the air-liquid interface using alveolar epithelial cells derived from human induced pluripotent stem cells
Published in Scientific reports (26-03-2020)“…Research on acute and chronic lung diseases would greatly benefit from reproducible availability of alveolar epithelial cells (AEC). Primary alveolar…”
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Human iPSC-Derived Retinas Recapitulate the Fetal CRB1 CRB2 Complex Formation and Demonstrate that Photoreceptors and Müller Glia Are Targets of AAV5
Published in Stem cell reports (14-05-2019)“…Human retinal organoids from induced pluripotent stem cells (hiPSCs) can be used to confirm the localization of proteins in retinal cell types and to test…”
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Defining Phenotype, Tropism, and Retinal Gene Therapy Using Adeno-Associated Viral Vectors (AAVs) in New-Born Brown Norway Rats with a Spontaneous Mutation in Crb1
Published in International journal of molecular sciences (30-03-2021)“…Mutations in the Crumbs homologue 1 ( ) gene cause inherited retinal dystrophies, such as early-onset retinitis pigmentosa and Leber congenital amaurosis. A…”
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CRB1 is required for recycling by RAB11A+ vesicles in human retinal organoids
Published in Stem cell reports (12-09-2023)“…CRB1 gene mutations can cause early- or late-onset retinitis pigmentosa, Leber congenital amaurosis, or maculopathy. Recapitulating human CRB1 phenotypes in…”
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18
Increased Presence of Monounsaturated Fatty Acids in the Stratum Corneum of Human Skin Equivalents
Published in Journal of investigative dermatology (01-01-2013)“…Previous results showed that our in-house human skin equivalents (HSEs) differ in their stratum corneum (SC) lipid organization compared with human SC. To…”
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Naturally occurring NOTCH3 exon skipping attenuates NOTCH3 protein aggregation and disease severity in CADASIL patients
Published in Human molecular genetics (21-07-2020)“…CADASIL is a vascular protein aggregation disorder caused by cysteine-altering NOTCH3 variants, leading to mid-adult-onset stroke and dementia. Here, we report…”
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20
Oncogenomic analysis of mycosis fungoides reveals major differences with Sézary syndrome
Published in Blood (01-01-2009)“…Mycosis fungoides (MF), the most common cutaneous T-cell lymphoma, is a malignancy of mature, skin-homing T cells. Sézary syndrome (Sz) is often considered to…”
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