Rate My Professor Stefan Pulst

Dr. Pulst's clinical and research interests focus on inherited diseases of the nervous system, emphasizing spinocerebellar ataxias, amyotrophic lateral sclerosis/frontotemporal dementia, and Parkinson’s disease. His laboratory investigates mechanisms of adult-onset neurodegeneration through genetic analysis of human pedigrees, cellular modeling, and mouse models. Studies examine RNA-binding proteins ATXN2 and STAUEN1 in autophagy, unfolded protein response under chronic stress, using transcriptomics, physiologic, and behavioral analyses. Key discoveries include identifying the SCA2 gene via CAG/polyglutamine expansion in ATXN2 (Pulst et al., Nature Genetics, 1996), mapping and solving SCA4 with ZFHX3 GGC-repeat expansion after 25 years (2024), developing antisense oligonucleotide therapy for SCA2, and a molecule diminishing alpha-synuclein deficits in Parkinson’s models. Additional contributions encompass the rat shaker gene and STAU1's role in RNA stress granules. Select publications are 'Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2' (1996), 'Spinocerebellar Ataxia Type 2' (Scoles DR, Pulst SM, Adv Exp Med Biol, 2018), and 'Staufen1 in Human Neurodegeneration' (Paul S et al., Annals of Neurology, 2021). Dr. Pulst leads the Pulst and Scoles Laboratories, received the NINDS Outstanding Investigator Award, and was elected AAAS Fellow in 2020.