A5013312105- Biomedical Research and Pathophysiology
- Renal and related cancers
- Neonatal Health and Biochemistry
- Renal Diseases and Glomerulopathies
- Amino Acid Enzymes and Metabolism
- Tissue Engineering and Regenerative Medicine
- Chronic Kidney Disease and Diabetes
- Chronic Lymphocytic Leukemia Research
- Erythrocyte Function and Pathophysiology
- Genetic and Kidney Cyst Diseases
- Neonatal Respiratory Health Research
- Renal cell carcinoma treatment
- Renal and Vascular Pathologies
- Birth, Development, and Health
- Methemoglobinemia and Tumor Lysis Syndrome
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Acute Lymphoblastic Leukemia research
- 3D Printing in Biomedical Research
- Digestive system and related health
- Cell Image Analysis Techniques
- Mesenchymal stem cell research
- Infant Nutrition and Health
- Lymphatic Disorders and Treatments
- Pluripotent Stem Cells Research
- Connective Tissue Growth Factor Research
Emma Kinderziekenhuis
2023-2025
Amsterdam University Medical Centers
2023-2025
University of Amsterdam
2024
KU Leuven
2014-2023
Organ Recovery Systems (Belgium)
2018
Catholic University of America
2015
Swiss Paraplegic Research
2015
Universidade Estadual Paulista (Unesp)
2009
The C-terminal variants G1 and G2 of apolipoprotein L1 (APOL1) confer human resistance to the sleeping sickness parasite Trypanosoma rhodesiense, but they also increase risk kidney disease. APOL1 APOL3 are death-promoting proteins that partially associated with endoplasmic reticulum Golgi membranes. We report in podocytes, either helix truncation (APOL1Δ) or deletion (APOL3KO) induces similar actomyosin reorganization linked inhibition phosphatidylinositol-4-phosphate [PI(4)P] synthesis by...
Preterm birth, defined as birth before the gestational age of 37 weeks, affects 11% newborns worldwide. While extensive research has focused on immediate complications associated with prematurity, emerging evidence suggests a link between prematurity and development kidney disease later in life. It been demonstrated that normal course is interrupted infants born prematurely, causing an overall decrease functional nephrons. Yet, pathogenesis leading to alterations subsequent...
Apolipoprotein-L1 (
Objectives Mesenchymal stem cells derived from human amniotic fluid (hAFSCs) are a promising source for cellular therapy, especially renal disorders, as subpopulation is the fetal urinary tract. The purpose of this study was to evaluate if hAFSCs with progenitor phenotype demonstrate nephroprotective effect in acute ischemia reperfusion (I/R) model and prevent late stage fibrosis. Methods A total 45 male 12-wk-old Wistar rats were divided into three equal groups;: subjected I/R injury...
Abstract The human ubiquitous protein cystinosin is responsible for transporting the disulphide amino acid cystine from lysosomal compartment into cytosol. In humans, Pathogenic mutations of CTNS lead to defective function, intralysosomal accumulation and development cystinosis. Kidneys are initially affected with generalized proximal tubular dysfunction (renal Fanconi syndrome), then disease rapidly affects glomeruli progresses towards end stage renal failure multiple organ dysfunction....
Cystinosis is a rare, incurable lysosomal storage disease caused by mutations in the CTNS gene encoding cystine transporter cystinosin, which leads to accumulation all cells of body. Patients with cystinosis display signs podocyte damage characterized extensive loss podocytes into urine at early stages, glomerular proteinuria, and development focal segmental glomerulosclerosis (FSGS) lesions. Although standard treatment cysteamine decreases cellular levels, it neither reverses injury nor...
In humans, nephrogenesis is completed prenatally, with nephrons formed until 34 weeks of gestational age. We hypothesized that urine preterm neonates born before the completion a noninvasive source highly potent stem/progenitor cells. To test this hypothesis, we collected freshly voided at day 1 after birth from 31–36 age and characterized isolated cells using single–cell RT-PCR strategy for gene expression analysis flow cytometry immunofluorescence protein analysis. Neonatal expressed...
Nephropathic cystinosis is an inherited autosomal recessive lysosomal storage disorder characterized by the pathological accumulation and crystallization of cystine inside different cell types. WBC determination forms basis for diagnosis therapeutic monitoring with depleting drug (cysteamine). The chitotriosidase enzyme a human chitinase, produced activated macrophages. Its elevation documented in several disorders. Although, about 6% Caucasians have deficiency due to homozygosity 24-bp...
Objective: To evaluate if the up-regulation of vascular endothelial growth factor strengthens protective effect amniotic fluid stem cells in a renal ischemia-reperfusion injury model. Design: Randomized animal study. Settings: University research laboratory. Subjects: A total 40 males 12-week-old Wistar rats were subjected to and assigned four groups: cells, factor-amniotic two different doses, vehicle. Ten animals used as sham-controls. Intervention: Six hours after induction injury, or...
The effects of experimental type 1 diabetes were investigated in the acinar epithelium rat ventral prostate, focusing on rates cell proliferation and frequency apoptosis p63-positive cells. Type was induced adult male Wistar rats by a single alloxan administration (42 mg/kg b.w.) its analysed for week 3 months after establishment disease. A group diabetic treated daily with 5 IU insulin during had been diagnosed. Immunocytochemical methods localization antigen (PCNA), androgen receptor (AR)...
Tissue engineering is a field in progressive expansion and requires constant updates methods devices. One of the central fields development biocompatible, biodegradable, injectable scaffolds, such as collagen microcarriers. To enhance cell attachment produce cost-effective culture solution with local stimulation cells, basic fibroblast growth factor (bFGF) or transforming factor-β1 (TGF-β1) was covalently immobilized on microcarriers either by 1-ethyl-3-(3-dimethylaminopropyl)...
Cystinosis is an autosomal recessive lysosomal storage disorder characterized by the defective transport of amino acid cystine out lysosome due to a deficiency cystinosin, transporter. Patients have accumulation in various tissues, leading cellular stress and damage, particularly kidney, cornea, other extrarenal tissues. Cysteamine, cystine-depleting agent, improves survival delays progression disease, but it does not prevent development either renal failure or complications. Furthermore,...
Alport syndrome (AS) is a genetic disorder involving mutations in the genes encoding collagen IV α3, α4 or α5 chains, resulting impairment of glomerular basement membrane. Podocytes are responsible for production and correct assembly isoforms; however, data on phenotypic characteristics human AS podocytes their functional alterations currently limited. The evident loss viable into urine patients with active disease enables isolation non-invasive way. Here we isolated, immortalized, subcloned...
During development, nephron structures are derived from a SIX2+ stem cell population. After 36 weeks of gestation, these cells exhausted, and no new nephrons formed. We have previously described non-invasive strategy to isolate expand the native kidney urine preterm neonates, named neonatal stem/progenitor (nKSPC). Here, we investigated safety feasibility administering nKSPC into human kidneys discarded for transplantation during normothermic machine perfusion (NMP) evaluated regenerative...
Messenger RNA (mRNA) therapies are emerging in different disease areas, but have not yet reached the kidney field. Our aim was to study feasibility treat genetic defect cystinosis using synthetic mRNA cell models and ctns-/- zebrafish embryos. Cystinosis is a prototype lysosomal storage disorder caused by mutations CTNS gene, encoding cystine-H+ symporter cystinosin, leading cystine accumulation all cells of body. The kidneys first most severely affected organs, presenting glomerular...
Apolipoprotein L1 (APOL1) high-risk genotypes (HRG), G1 and G2, increase the risk of various non-diabetic kidney diseases in African population. To date, precise mechanisms by which APOL1 variants induce injury on podocytes other cells remain unclear. Trying to unravel these mechanisms, most studies have used animal or cell models created gene editing. We developed characterised conditionally immortalised human podocyte lines derived from urine a donor carrying HRG G2/G2. Following induction...
Nephropathic cystinosis is an inherited lysosomal storage disorder caused by pathogenic variants in the cystinosin (CTNS) gene and characterized excessive shedding of proximal tubular epithelial cells (PTECs) podocytes into urine, development renal Fanconi syndrome end-stage kidney disease (ESKD). We hypothesized that compensation for cell losses, kidneys undertake a regenerative effort, searched presence progenitor (KPCs) urine patients. Urine was cultured specific medium to isolate...