ALS Compendium

Title: The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS

Year Published: 2013

Observation: found high molecular weight DPR aggregates in 3 reading frames. found that most inclusions contain poly-(Gly-Ala) and, to a lesser extent, poly-(Gly-Pro) and poly-(Gly-Arg) dipeptide-repeat proteins presumably generated by non-ATG–initiated translation from the expanded GGGGCC repeat in three reading frames

Comment: Non ATG innitiated translation of GGGGCC repeat expansion leads to DPR aggregates

Citation: Mori, K., Weng, S.-M., Arzberger, T., May, S., Rentzsch, K., Kremmer, E., … Edbauer, D. (2013). The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS. Science (New York, N.Y.), 339(6125), 1335–8. doi:10.1126/science.1232927

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