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Gene Synthesis> | … The plasmids encoding the DPRs GA, GR, PR and PA, all containing 50 repeats and a C-terminal FLAG tag, as well as the Pur-alpha deletion constructs, all containing a C-terminal FLAG tag, were synthesized (Genscript, Piscataway, USA) and subcloned into the pCMV6-Entry … | Get A Quote |
The exact mechanism underlying amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) associated with the GGGGCC repeat expansion in C9orf72 is still unclear. Two gain-of-function mechanisms are possible: repeat RNA toxicity and dipeptide repeat protein (DPR) toxicity. We here dissected both possibilities using a zebrafish model for ALS. Expression of two DPRs, glycine-arginine and proline-arginine, induced a motor axonopathy. Similarly, expanded sense and antisense repeat RNA also induced a motor axonopathy and formed mainly cytoplasmic RNA foci. However, DPRs were not detected in these conditions. Moreover, stop codon-interrupted repeat RNA still induced a motor axonopathy and a syner... More