Kolokviji IJS, sreda, 25. 2. 2026, ob 13:00, prof. dr. Edor Kabashi

[Martin Klanjsek]

Vabimo vas na 7. predavanje iz sklopa »Kolokviji na IJS« v akademskem letu 2025/26, ki bo v sredo, 25. februarja 2026, ob 13:00 v Veliki predavalnici Instituta »Jožef Stefan« na Jamovi cesti 39 v Ljubljani. Predavanje bo v angleščini. Na voljo bodo kava, pijača in prigrizki.

 

 

Od Charcota do zebraste ribice: razkrivanje ALS, gen za genom

 

prof. dr. Edor Kabashi

Imagine – Institut za genetske bolezni in Institut Charcot, Pariz, Francija

 

 

Zebraste ribice so se izkazale kot izjemno učinkoviti modeli za proučevanje genetskih osnov amiotrofične lateralne skleroze (ALS) in sorodnih nevroloških bolezni. Njihov hiter razvoj, prosojno telo ter prisotnost ohranjenih ortologov večine z ALS povezanih genov raziskovalcem omogočajo, da v realnem času spremljajo potek bolezni. Z izklapljanjem posameznih genov ali z vnosom človeških mutacij, povezanih z ALS, je mogoče ustvariti širok nabor modelov zebraste ribice, ki posnemajo ključne značilnosti bolezni. V predavanju bom predstavil, kako ti modeli omogočajo neposredne primerjave med različnimi z ALS povezanimi geni in razkrivajo njihove skupne mehanizme. V modelih z mutacijami v genih C9orf72, TDP-43, FUS in TBK1 opažamo tako skupne kot tudi različne patološke poti, kar pomaga pojasniti, zakaj se ALS med posamezniki tako različno izraža. Te raziskave obenem razkrijejo več obetavnih terapevtskih tarč, od katerih nekatere že napredujejo v klinično testiranje. Predstavil bom tudi Institut Charcot, novo pobudo, ki povezuje vodilne francoske klinične in raziskovalne skupine na področju ALS. Njegovo poslanstvo je pospešiti pot od laboratorijskih odkritij do učinkovitih terapij za ljudi z ALS.

 

O predavatelju: Edor Kabashi je direktor in ustanovni član Instituta Charcot, virtualnega instituta, posvečenega raziskovanju ALS, ter vodja laboratorija na Institutu Imagine, bolnišnica AP‑HP Necker, Univerza Sorbonne, v Parizu v Franciji. Je prejemnik projektov ERC Consolidator kot tudi ERC Proof of Concept.

 

 

Napovednik predavanja in posnetke preteklih predavanj najdete na spletni strani »Kolokvijev na IJS«  <https://kolokviji.ijs.si/> https://kolokviji.ijs.si/, posnetke pa tudi posebej na  <https://video.arnes.si/?q=Kolokviji_IJS> https://video.arnes.si/?q=Kolokviji_IJS (zadnje štiri sezone) in  <http://videolectures.net/kolokviji_ijs> http://videolectures.net/kolokviji_ijs (starejši).

 

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We invite you to the 7th lecture in the framework of »JSI Colloquia« in the academic year 2025/26, which will take place on Wednesday, February 25, 2026, at 1:00 PM in the main lecture hall of Jožef Stefan Institute, Jamova cesta 39 in Ljubljana. The lecture will be held in English. Coffee, drinks and snacks will be available.

 

 

>From Charcot to zebrafish: decoding ALS one gene at a time 

 

Prof. Dr. Edor Kabashi

Imagine – Institute of Genetic Diseases and Charcot Institute, Paris, France

 

 

Zebrafish have emerged as remarkably powerful models for exploring the genetic roots of amyotrophic lateral sclerosis (ALS) and related neurological disorders. Their rapid development, transparent bodies and the presence of conserved counterparts to most ALS-associated genes allow researchers to watch disease processes unfold in real time. By switching off specific genes or introducing human ALS-linked mutations, it is possible to create a wide range of zebrafish models that mimic key features of the disease. In this talk, I will present how these models enable direct comparisons between different ALS-associated genes and reveal the mechanisms they share. Across models carrying mutations in C9orf72, TDP-43, FUS, and TBK1, we observe both common and distinct pathways that drive disease — insights that help explain why ALS can vary so widely from one patient to another. These studies have also uncovered several promising therapeutic targets, some of which are already advancing through clinical testing. I will also introduce the Charcot Institute, a new initiative uniting France's leading ALS clinicians and researchers. Its mission is to accelerate the journey from laboratory discoveries to effective treatments for people living with ALS.

 

About the lecturer: Edor Kabashi is the director and a founding member of the Charcot Institute, a virtual institute dedicated to advancing ALS research, and a laboratory director at the Imagine Institute, AP-HP Necker Hospital, Sorbonne University, in Paris, France. He is the recipient of an ERC Consolidator Grant and an ERC Proof of Concept Grant.

 

 

The lecture trailer and recordings of past lectures can be found on the website of »JSI colloquia«  <https://kolokviji.ijs.si/> https://kolokviji.ijs.si/, and recordings specifically at  <https://video.arnes.si/en/?q=Kolokviji_IJS> https://video.arnes.si/en/?q=Kolokviji_IJS (last four seasons) and  <http://videolectures.net/kolokviji_ijs> http://videolectures.net/kolokviji_ijs (older).

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