SNC'21 Programme Preview and Highlights

SNC'21 Plenary and Special Lectures

Sonia M. Vallabh

Dr. Sonia M. Vallabh will be the SNC'21 presenter of the A. O. Župančič Memorial Lecture, entitled Genetic prion disorders and patient-scientist's mandate on Friday, September 24 at 19:00. The lecture will serve two functions: it will conclude the Educational Workshop on Proteostasis and Protein Misfolding in CNS Disorders and also open the Neuroscience and Society Dialogue event, for the general public.

Dr. Vallabh describes her work like this: "The goal of the lab that I co-run with my husband Eric Minikel is to advance a therapeutic for prion disease, a currently untreatable rapid neurodegenerative disease. Over the years we've come to appreciate that this goal requires that we do many different kinds of science. We perform preclinical studies in animals, but also biomarker and natural history studies to help support future clinical trials, genetics to illuminate who is at risk, detailed molecular biology to understand how potential therapeutics are working, and a lot of model and assay development to make sure we can ask all of the right questions." She adds: "Our goal is oriented by the fact that I am personally at risk for genetic prion disease. So from this perspective, my answer will have a translational bias. I'm very excited by the growing evidence that lowering the amount of prion protein in the brain is an effective way to treat prion disease, or even delay or prevent it if treatment is initiated early enough. We've had genetic proof of concept for this therapeutic hypothesis for a long time, but now we are at a moment in time where we can finally test it with existing therapeutic technologies. There is much work to do, but I hope we are beginning to glimpse a future in which prion disease is treatable or even preventable. Another very exciting development is the relatively recent development and clinical implementation of RT-QuIC, an excellent diagnostic assay which I hope will help us identify prion disease patients sooner. In such a rapid disease, early diagnosis will be key to being able to help patients once we do have useful therapeutics to offer."

Regarding advice to an aspiring young neuroscientist, Dr. Vallabh states: "I once heard a researcher say, "Research is for the next generation," alluding to the length of the drug development process. I understand where they were coming from, but in this moment in time, I find that I don't agree. We live in an amazing biotechnological moment, particularly for genetically well-defined diseases. I would say, if you are really committed, don't underestimate what can be done, even in our lifetimes!"

Eiko Fried

Professor Eiko Fried will deliver his plenary talk entitled Systems all the way down: studying mental health problems as biopsychosocial systems on Saturday, September 25 at 12:00. Here are his answers to our three short questions:

Q: What is the focus of your research?

A: My interests are how to best understand, measure, model, and classify mental health problems. To do so, I conceptualize mental health or illness as emergent properties that arise from complex, dynamical, biopsychosocial systems, rather than as clear-cut categories with simple causes. I work at the intersection of clinical psychology, psychiatry, epidemiology, methodology, and complexity science, and enjoy learning about philosophy of science. Besides that, I am interested in improving cumulative psychological science through open science practices.

Q: What is currently the most promising development in your research field?

A: I am excited about formalizing theories as computational models, which can then be used to test how data would look like if our theories were true. Such models are in many ways superior to narrative, ambiguous theories that are common in our field and vulnerable to many hidden assumptions and unknowns.

Q: What advice would you give to a young (neuro)scientist?

A: I have learned a lot from inter-disciplinary collaborations, and can only recommend creating dialogues with researchers in adjacent areas.

Tracy Bale

Professor Tracy Bale is a Professor of Pharmacology and Director of the Center for Epigenetic Research in Child Health and Brain Development in the School of Medicine at the University of Maryland, Baltimore, USA.

Her research focuses on understanding the role of stress dysregulation in neurodevelopmental and neuropsychiatric diseases, and the sex differences that underlie disease vulnerability in humans. She is interested in developing models of parental stress and the germ cell involvement in intergenerational programming of neurodevelopment.

She serves on many internal and external advisory committees, panels, and boards and served as Chair of the NNRS CSR study section and was a Reviewing Editor at the Journal of Neuroscience. She was awarded the Top 100 Women in Maryland 2020 title.

Professor Bale is also the President of the International Brain Research Organisation, IBRO. We asked her what seperates the mission of IBRO from the missions of other well known neuroscience organizations like Federation of European Neuroscience Societies and Sociaty for Neuroscience of the USA. Professor Bale responded: "IBRO is international and supports neuroscience training and programs around the globe." When asked if the role of IBRO has changed in the world that has already had a Decade of the Brain, some 20 annual Brain Awareness Weeks, and hefty investments into reuroscience through endeavors like the Human Brain Project, Professor Bale said: "Certainly - IBRO is constantly working to reevaluate itself and determine what the changing needs are for neuroscience within all our regions."

Professor Bale will give her plenary talk entitled Extracellular vesicles as stress signals: Identifying novel mechanisms of neurodevelopmental programming on Saturday, September 25, 2021 at 17:00 CET. Her advice to young neuroscientists is to "think outside the box and be creative".

Giuseppe Lauria

Professor Guiseppe Lauria from the University of Milan and IRCCS Foundation "Carlo Besta" Neurological Institute will present the Dr. Janez Faganel Memorial lecture entitled Small fiber neuropathy on Friday, September 24, 2021 at 12:00. This will be the opening talk of the Ljubljana Clinical Neurophysiology Symposium 2021.

Knowledge on small fibre neuropathy has been widened by new preclinical and clinical knowledge leading to a more comprehensive approach to patients in clinical practice and research. The spectrum of clinical features has been widened from the classical presentation of burning feet as length-dependent small fibre neuropathy to that of small fibre dysfunction and/or degeneration associated with focal, diffuse and episodic neuropathic pain syndromes. The involvement of small nerve fibres in neurodegenerative diseases has been further defined, challenging the relationship between neuropathic pain symptoms and small fibre loss. New studies expanded the panel on genes involved in small fibre neuropathy and opened new avenues for personalised therapies.

Dr. Isabelle Budin-Ljøsne will preside over the final symposium of the SNC'21, devoted to Brain health across the lifespan - Finfings from the Lifebrain project. The symposium will take place on Saturday, September 25, 2021, at 15:00 CET. In the attached short video, Dr. Budin-Ljøsne introduces the concept of brain health and shares her approach to keeping a healthy brain.

Educational Workshop on Protein Misfolding in CNS Disorders

Professor Rick Morimoto will open the Educational Workshop with his talk Proteostasis Collapse: A Basis for Aging and Neurodegenerative Diseases on Friday, September 24 at 15:00 CET.

Professor Gabor Kovacs will provide An update on Tau-related diseases in which he will present a neuropathology-based approach to tau-related conditions and highlight current hot topics of the field on Friday, September 24 at 15:45 CET.

Boris Rogelj

Professor Boris Rogelj is Head of Department at Jozef Stefan Institute, Ljubljana. He is a molecular biologist/biochemist researching the molecular processes underlying neurodegenerative diseases, focusing on the pathological RNA-protein interactions and mechanisms underlying amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTD). He will give his lecture on Friday, September 24, at 16:15 CET. Prof. Rogelj kindly answered a few of our questions:

Q: Can you tell us a bit more about the focus of your research?

A: Proteins TDP-43 and FUS are major contributing factors in ALS and FTD. I was part of the team that identified mutations in genes coding for TDP-43 and FUS in ALS. They are both predominantly nuclear proteins that are implicated in processing and transport of RNA and in disease they missacumulate in the cytoplasm and form pathognomonic aggregates. Thus, our work has been focusing on the nuclear transport and RNA binding properties of TDP-43 and FUS as well as mechanisms leading to aggregation. We have also been working on the genetics, pathology and molecular mechanisms of the hexanucleotide repeat expansion mutation in C9ORF72 gene. We have shown that the sense strand can form noncanonical RNA/DNA structures G-quadruplexes, while the antisense strand can form i-motifs/protonated hairpins. Our current work involves studies of the interactome and toxicity of C9orf72-associated RNA repeats and dipeptide repeats (DPRs) translated from these RNAs. We have characterized the proteins that bind to the sense strand RNA and form paraspeckle-like RNA foci. We have also recently shown that some pathological accumulation of some DPRs may have a direct effect on proteostasis.

Q: What do you consider the most exciting and promising development in your field?

A: The discovery of the C9ORF72 mutation 10 years ago has made important shifts not only in understanding some of the mechanisms leading to ALS and FTD, but also wider in the biomedical and biological fields. The mutation is extremely challenging to study as it is connected to many basic and a few novel concepts and mechanisms, such as noncanonical nucleic acid structures, repeat associated non-ATG translation, liquid-liquid phase transitions, etc. Also there are extremely promising developments in therapeutical approaches such as antisense oligonucleotides for treatment and viral vectors for delivery.

Q: What advice would you give to someone considering a career in neuroscience?

A: Science in general is not just a job but a way of life.

Professor Roger A. Barker will address the latest advances in Huntington disease research on Friday, September 24, 2021 at 17:15 CET

Professor Patrik Brundin has entitled his lecture Now it is time for research to crack Parkinson's disease. He will present his arguments on Friday, September 24, 2021 at 16:45 CET

Professor Adriano Aguzzi will review the current knowledge on the infectivity, neurotoxicity and neuroinvasiveness of prions, address the challenge posed to medicine by prion strains, and showcase some of his laboratory's recent findings regarding prion biology. Tune into his presentation on Friday, September 24th, 17:45-18:15 CET.

Professor Holger Wille will offer A structural biologist's view of neuroscience that has led his group to create a structure-based prion vaccine on Friday, September 24 at 18:15 CET.