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세미나
세미나

Pathogenesis studies of neurodegenerative polyglutamine diseases

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  • 2015-11-13

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[2015 Fall Life Sciences & IBB Regular Seminar]


             


                


            ▶Subject: Pathogenesis studies of neurodegenerative polyglutamine diseases


              


            ▶Speaker: Prof. Janghoo Lim (Yale University School of Medicine)


                      


            ▶Date: 4:00PM/Nov./13(Fri.)/2015


               


            ▶Place: Auditorium(1F), Postech Biotech Center


              


                    *Abctract


                  The main research goal of my laboratory is to better understand the molecular and cellular mechanisms that are responsible for neurodegeneration and ultimately to translate our findings into the development of therapeutics for neurodegenerative diseases. To achieve this goal, we focus on polyglutamine diseases as model systems. Polyglutamine diseases are dominantly inherited neurodegenerative conditions caused by an expansion of a CAG trinucleotide repeat encoding a glutamine tract in the respective disease-causing proteins. Polyglutamine expansion makes the host protein toxic, resulting in the formation of mutant protein aggregates and cell death. The commonalities in the nature of these mutations and the presentation of the different polyglutamine disorders suggest the occurrence of a common pathogenic mechanism. Such mechanism, however, has remained elusive and to date there are no cures or even effective therapies for most of these diseases. We have been focusing on two distinct polyglutamine diseases, named spinocerebellar ataxia type 1 (SCA1) and spinal and bulbar muscular atrophy (SBMA). SCA1 is a dominantly inherited disease characterized by the progressive degeneration of neurons, specifically those in the cerebellum and brainstem. SBMA is an X-linked progressive neuromuscular disease. SBMA patients present with progressive weakness and muscle atrophy resulting from the degeneration of the motor neurons and skeletal muscles. We have recently identified Nemo-Like Kinase (NLK) as a genetic modifier in the pathogenesis of SCA1 and SBMA and thus as a novel putative therapeutic target for those diseases. By utilizing a variety of experimental approaches, including biochemistry, cell biology, and Drosophila and mouse genetics, we showed that reducing NLK expression lowers the severity of protein toxicity in SCA1 and SBMA. The finding that NLK and its signaling pathway affect the activity of two distinct polyglutamine diseases provide evidence for the occurrence of common pathogenetic mechanisms in this group of neurodegenerative diseases.


                 


           ▶Inquiry: Prof. Yoontae Lee (279-2354) 


       


              * This seminar will be given in English.


          please refrain from taking photos during seminars. *



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