Projects
Design, synthesis and screening of tRNA stabilising and antigenomic compounds for the treatment of mitochondrial diseases. Ghent University
Mitochondria are referred to as the powerhouse or batteries of the cell, this is because they
produce energy for the cell. To generate this energy (ATP) mitochondria build their own machinery,
using mitochondrial DNA (mtDNA) as a blueprint. Errors in the mtDNA are rare, but do occur, which
can lead to severe and progressive diseases often with fatal outcome. There is currently no cure for
...
Mitochondrial DNA content in cardiovascular ageing and disease: a population study. KU Leuven
A major burden of modern society is the progressive increase in age-associated diseases such as heart failure (HF). The genetic and environmental drivers of left ventricular (LV) dysfunction progression remain to be elucidated. LV dysfunction is associated with changes in cardiac energy metabolism. Mitochondria play a central role in a variety of cardiac cell functions. The mitochondrial DNA (mtDNA) content correlates with the size and number ...
Endothelial mitochondria: enigmatic function and potential target for tumor anti-angiogenesis? KU Leuven
Blood vessel formation (angiogenesis) promotes tumor growth and metastasis. Current antiangiogenic therapies target endothelial growth factors, but suffer from limited efficacy. Also, since tumor vessels are structurally and functionally abnormal (which impairs perfusion), pruning them by anti-angiogenic therapy may create a more hostile nutrient-deprived milieu, promoting cancer cell escape and dissemination. A novel paradigm is to ...
Mitochondrial homeostasis and its importance for neurodegenerative disorders. KU Leuven
Parkinson Disease is the second most important neurodegenerative disorder. Research has shown that mutations in a particular gene (PINK1) can cause the disease by affecting the mitochondria, the energy factories of the brain. PINK1 is cleaved by a molecular scissor, PARL, which is sitting in the mitochondria. A second protein, called PGAM5 is also cleaved by PARL. We think that these three proteins together are very important to maintain the ...
'The role of ATP13A2 in mitochondrial functionality' KU Leuven
ATP13A2 is a late endolysosomal P5B-type transport ATPase that exports polyamines from the late endolysosome to the cytosol. Loss-of-function mutations in this transporter lead to a range of neurodegenerative disorders, which are all characterized by increased oxidative stress and a flawed mitochondrial-lysosomal axis at the cellular level. In addition, overexpression of ATP13A2 in cell models has been reported to provide protection against ...
Identification of proteases that activate membrane-bound transcription factors during mitochondrial retrograde regulation Ghent University
Plants are our main food and feed source, but they often get threatened by adverse environments in which they must adapt to survive. These adverse environments due to (a)biotic factors cause major yield losses, that are expected to become worse due to climate change, raising the demand for stress tolerant crops. Mitochondria contain their own genome whose expression needs to be coordinated with the nuclear genome. This resulted in an ...
Development of a novel diagnotic assay for the biochemical diagnosis of mitochondrial disorders in vital tissue KU Leuven
Primary mitochondrial disorders are characterized by aberrancies in normal mitochondrial functioning due to mutations in either nuclear or mitochondrial DNA-encoded genes. The estimated prevalence is at least 1 in 5.000 births, suggesting that there are at least 1.200 individuals in Flanders with an inherited mitochondrial disorder. However, less than 250 patients in Flanders have a diagnosis. We follow approximately 100 patients with a ...
Endoplasmic reticulum and mitochondria: common drivers of human Ī² cell and neuronal fate KU Leuven
Diabetes and brain disorders appear at first glance to be completely different diseases. However, human
genetics has unveiled some severe forms of diabetes in babies that are associated with abnormal brain
development, including small brain size and epilepsy. Intriguingly, the mutations causing these diseases hit
genes with a role in two critical organelles: the endoplasmic reticulum and mitochondria, which together control ...
Mitochondrial modulation of healthy aging through redox-mediated and peptidergic signaling. KU Leuven
The mitochondria are inextricably linked to the process of aging. Whether through the release of reactive oxygen species (ROS), peptidergic signals or otherwise, mitochondrial signaling can potently enhance lifespan and health in a multitude of species. Despite the mitochondriaās apparent and central role in modulating aging, many open questions remain on the subject of how this signaling exactly occurs. For example, many nodes in the ...