Stem cell disease modelling to investigate mechanisms of selective vulnerability of motor neurons

Motor neurons are amongst the longest neurons in the body with its axon extending up to a metre to connect target muscle. Accumulating evidence suggests independent pathomechanisms affect distinct cellular compartments of motor neurons – soma and axon – leading to motor neuron loss and axon degeneration, respectively.

The overarching aim of our research is to decipher the molecular pathomechanisms leading to selective vulnerability of motor neurons as observed in motor neuron disease. In addition, we also focus on understanding and exploit mechanisms that control axonal homeostasis to promote axonal maintenance and preserve axons from degeneration. To achieve this we use cutting edge technologies like human stem cell modelling including 3D organoid models, CRISPR/Cas9 genome editing and human post-mortem studies.