Muela Lab

Research focus

ERC Starting Grant · 2018

Molecular mechanisms underlying selective neuronal death in motor neuron diseases

The mechanisms behind neuronal death in different motor neuron diseases (MND) remain unknown. These MNDs include the devastating spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS). A fascinating question in neurodegeneration research is why mutations in ubiquitously expressed genes result in the selective death of a specific neuronal subtype. The ubiquitously expressed and conserved survival of motor neuron (SMN) protein receives its name because its deficit results in MN degeneration. However, SMN known functions -spliceosome assembly and axonal mRNA transport- do not explain the selective MN vulnerability. Accumulation of intracellular aggregates in neurons is a…

From the public funding record at EU CORDIS. Describes the funded project, not the reviews below.