Orengo Lab

The Orengo Lab studies motor neuron degeneration using single gene mutation models to determine the common underpinnings involved in triggering this degeneration. Spinocerebellar ataxia type 1 (SCA1) is a devastating neurodegenerative disease characterized by progressive ataxia due to cerebellar degeneration. While the hallmark of SCA1 is loss of motor coordination or ataxia, as the disease progresses premature death occurs from complications secondary to bulbar dysfunction (breathing and swallowing difficulties). SCA1 is caused by expansion of CAG repeats encoding poly-glutamines (polyQ) in ATAXIN1 (Atxn1). We use a cross species approach of modeling disease in mice, zebrafish and human cell lines to find share pathways of pathology leading to motor neuron disease.