Projects
Untangling the pathophysiology of congenital disorders of glycosylation affecting the OST complex KU Leuven
Congenital disorders of glycosylation (CDG) are a group of over 100 inherited disorders characterised by defective glycosylation of proteins and lipids. Although the phenotypic and genetic characteristics of CDG as a whole are well established, their pathophysiology is poorly understood. In addition, the phenotype of affected patients is extremely variable, with dramatically different clinical presentations often appearing in patients with ...
Training the future generation of clinical and basic reseachers for Congenital Disorders of Glycosylation KU Leuven
Customized glycosylation engineering to enhance lysosomal enzyme delivery to muscle tissue in congenital and acquired lysosomal dysfunction. Ghent University
Our laboratory has pioneered the design of yeast-based expression systems for the production of biopharmaceuticals that can be modified with endolysosome-targeting glycans (glycans highly substituted with mannose-6-phosphate). Furthermore, we have recently developed the GlycoDelete technology, which can convert N-glycans in pharmacokinetics-neutral small structures and which at the same time leads to enhanced secretion of lysosomal enzymes ...
Role of ARF6 in PSEN1 mediated endo-lysosomal defects Flanders Institute for Biotechnology
Glycosylatie van angiogene factoren via de hexosamine biosyntheseweg, een nutriënten-sensor met een functie in metabole signalering in angiogenese? KU Leuven
Blood vessels ensure nutrient and oxygen supply to all cells via transport through the endothelium. This endothelial cell (EC) layer lining the luminal side of blood vessels forms a selective interface between the circulating blood and the surrounding tissue. ECs display high plasticity: i.e. in health, ECs in established blood vessels are quiescent and rarely divide, but upon stimulation in pathological conditions (ischemic, inflammatory or ...
Biochemical and biological domain functions of the human MMP-9 propeptide KU Leuven
Proteases are enzymes catalysing the breakdown of proteins into polypeptides or single amino acids, a process called proteolysis. They are key molecules involved in different physiological processes, ranging from digestion of food proteins to development and host protection against pathologies. However, they also contribute to pathological processes including autoimmune diseases and cancer. Matrix metalloproteinases (MMPs) are an important ...
Dolichol: An overlooked lipid in human health and disease KU Leuven
Genes encode proteins, that is a central dogma. However, many of these proteins need further tailoring to become fully functional. Glycosylation - the addition of ‘sugar trees’ to proteins - is one of the major modifications that occur in biology. The glycosylation pathway looks like an assembly line, with hundreds of workers and spare parts. By searching for novel genes in patients with a Congenital Disorder of Glycosylation (CDG) we ...
METABOLOMIC PROFILING OF PATIENTS WITH INBORN ERRORS OF METABOLISM KU Leuven
Glycosylation is an essential metabolic process of building sugar chains and adding them to organic molecules, which happens across all cell types. Defects in glycosylation lead to conditions called Congenital Disorders of Glycosylation (CDG). So far, more than 100 CDGs have been identified, but most of them without effective therapy. In addition, other disorders can lead to secondary CDGs (e.g. galactosemia). Recently, my co-promoter has ...