Van Den Bosch Lab

This year the ALS League, comes with a new challenge for De Warmste Week! One with which we can mark one of our points on our Bucket List!

Genetische wijzigingen in het C9orf72-gen worden in verband gebracht met de hersenziektes frontotemporale dementie en ALS. Onderzoekers verbonden aan VIB, KU Leuven en UZ Leuven melden nu in het vakblad JAMA Neurology dat een specifieke type PET-scan hersenveranderingen kan waarnemen bij mensen met een gewijzigd C9orf72-gen, nog vooraleer ze ziek worden. Volgens prof. Philip Van Damme, die aan het hoofd staat van het onderzoeksteam, suggereert dit dat de beeldvormingstechniek vroege veranderingen kan waarnemen jaren voordat de ziekte begint.

Our second example of the intense exchange of our basic science researchers with the Discovery Sciences team leads us into the field of neurology and peripheral neuropathies. More specifically to Charcot-Marie-Tooth disease (CMT), an incurable progressive weakening and atrophy of distal limb muscles. Patients develop foot deformities and gait difficulties, and also experience sensory abnormalities. CMT has a severe impact on the quality of life of its patients. Ludo Van Den Bosch, who is specialized in CMT, is professor neurobiology at the VIB-KU Leuven Center for Brain & Disease Research. 

Each year, the EU launches calls for postdoc research fellowships through the Marie Sklodowksa-Curie Actions. Fellowships provided through these actions support researchers regardless of age and nationality. Researchers working across all disciplines are eligible for funding. The prestigious two-year funding of Marie Curie fellowships attracts an enormous number of applications. Consequently, these fellowships are very competitive with a typical success rate of around 12%. VIB, however, manages to maintain a success rate of around 27%. The standing of these fellowships also provides an excellent springboard for the launch of an outstanding research career.
 

In a recent study in Molecular Neurodegeneration, researchers from VIB-KU Leuven led by prof. Philip Van Damme, reveal neuroprotective effects of progranulin against TDP-43 accumulation and neurodegeneration. The findings suggest that progranulin treatment may not only be of interest to certain patients with frontotemporal dementia, but also to patients with ALS.

The mutated and aggregated protein FUS is implicated in two neurodegenerative diseases: amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Using a newly developed fruit fly model, researchers led by prof. Ludo Van Den Bosch (VIB-KU Leuven) have zoomed in on the protein structure of FUS to gain more insight into how it causes neuronal toxicity and disease.

Inhibiting HDAC6 improves the structural stability of cells and protects against neuronal damage. Leuven research uncovered that targeting this mechanism could be a promising therapeutic approach for peripheral neuropathies, whether due to Charcot-Marie-Tooth disease (CMT) or as a side effect of chemotherapy.

​A team of researchers at VIB and KU Leuven used stem cell technology to generate motor neurons from ALS patients carrying mutations in FUS. They found disturbed axonal transport in these motor neurons, but also identified genetic and pharmacological strategies that mitigate these defects in cells.

A research team with Ludo Van Den Bosch (VIB-KU Leuven Center for Brain and Disease Research) at its helm has identified a new process that leads to the neurodegenerative brain diseases amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). The research uncovered important intra-cellular processes that lead to protein clumping in patients with defects in their C9orf72 genes. These key insights could lead to new treatments that prevent proteins from solidifying. This ground-breaking science would have never been possible, however, without close collaboration across VIB labs, core centers and national boundaries. We asked these VIB researchers to tell us more about the collaboration.