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In JoVE (1)
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Articles by Kishanda Vyboh in JoVE
JoVE General
वायरल शाही सेना के प्रतिदीप्ति द्वारा जांच
1Lady Davis Institute for Medical Research, Sir Mortimer B. Davis Jewish General Hospital, 2Department of Microbiology and Immunology, McGill University, 3Department of Medicine, Division of Experimental Medicine, McGill University
एक प्रतिदीप्ति
Other articles by Kishanda Vyboh on PubMed
Expanded ATXN3 Frameshifting Events Are Toxic in Drosophila and Mammalian Neuron Models
Spinocerebellar ataxia type 3 (SCA3) is caused by the expansion of the coding CAG repeat in the ATXN3 gene. Interestingly, a -1 base-pair frameshift occurring within an (exp)CAG repeat would henceforth lead to translation from a GCA frame, generating polyalanine stretches instead of polyglutamine. Our results show that transgenic expression of (exp)CAG ATXN3 led to -1 frameshifting events, which are deleterious in Drosophila and mammalian neurons. Conversely, transgenic expression of polyglutamine-encoding (exp)CAA ATXN3 was not toxic. Furthermore, (exp)CAG ATXN3 mRNA does not contribute per se to the toxicity observed in our models. Our observations indicate that expanded polyglutamine tracts in Drosophila and mouse neurons are insufficient for the development of a phenotype. Hence, we propose that -1 ribosomal frameshifting contributes to the toxicity associated with (exp)CAG repeats.
