The Pulst lab presented their work at three different Gordon Research Conferences this past summer. Gordon Research Conferences bring together clinicians, trainees, and renowned scientists from both academia and industry to present their pre-published research and discuss the future of the field.
Dr. Collin Anderson gave a talk and presented a poster at the Gordon Cerebellum Conference in Les Diablerets, Switzerland. Collin discussed deep brain stimulation and ataxia from two perspectives: first, pre-clinical results regarding the use of a novel form of deep brain stimulation in the deep cerebellum to reduce ataxic motor symptoms, and second, computational results regarding the optimization of deep brain stimulation parameters in other disease states to avoid ataxia as a side effect. Collin's first set of results was recently published in Annals of Neurology, and the second set of results is currently under review for publication.
Dr. Mandi Gandelman attended the Gordon Research Conference on Amyotrophic Lateral Sclerosis and Related Motor Neuron Diseases in Vermont and presented a poster on her work on STAU1 as a therapeutic target for ALS. Dr. Gandelman has found novel ways to reinstate proper function of the endoplasmic reticulum in neurons, a vital cellular organelle in charge of protein folding and calcium homeostasis. Dr. Gandelman’s experiments show that lowering STAU1 levels in neurons restores normal signaling between the endoplasmic reticulum and the nucleus, preventing neurons from activating death pathways.
This finding supports the overarching work being done at the Pulst lab developing therapies that target STAU1 for ALS as well as multiple other neurodegenerative diseases characterized by abnormal STAU1 function.
Dr. Stefan Pulst gave a keynote address at the Gordon Research Conference in Il Chiocco, Italy. This GRC was focused on Polyglutamine Diseases. His talk was entitled “Staufen-1 (STAU1) in neurodegeneration” and highlighted the role of the protein STAU1 during disease of the nervous system and how we can create therapies to control its harmful effects.
For more information:
Visit the Pulst Lab website:
http://pulstlab.genetics.utah.edu/index.html
Read Dr. Gandelman’s latest work on prevention of neuronal death:
Mandi Gandelman, Warunee Dansithong, Karla P Figueroa, Sharan Paul, Daniel R Scoles, Stefan M Pulst
Staufen 1 amplifies pro-apoptotic activation of the unfolded protein response.
bioRxiv 820225; doi: 10.1101/820225. https://www.biorxiv.org/content/10.1101/820225v1.article-metrics
Read Dr. Anderson’s work on treatment for ataxic symptoms:
Anderson CJ, Figueroa KP, Dorval AD, Pulst SM.
Deep cerebellar stimulation reduces ataxic motor symptoms in the shaker rat.
Ann Neurol. 2019. May;85(5):681-690. doi: 10.1002/ana.25464. https://www.ncbi.nlm.nih.gov/pubmed/30854718