My main research interest is the biological role of mRNA transport and local translation in neurons and their dysfunction in neurological diseases. Our emphasis is on the axonal function of the spinal muscular atrophy (SMA) disease protein SMN and the amyotrophic lateral sclerosis (ALS) disease protein TDP-43 in motor neurons. SMA results from reduced levels of the survival of motor neuron (SMN) protein, which has a well characterized function in spliceosomal small nuclear ribonucleoprotein assembly. ALS is characterized by cytoplasmic inclusions containing the RNA-binding protein TDP-43, and mutations in the gene encoding TDP-43 have been directly linked to the development of the disease. Currently, it is not understood how deficiency in proteins with a housekeeping function in RNA metabolism leads to the selective degeneration of motor neurons.