Proline/arginine dipeptide repeat polymers derail protein folding in amyotrophic lateral sclerosis

Proline/arginine dipeptide repeat polymers derail protein folding in amyotrophic lateral sclerosis

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The most frequent cause of familial Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD) are hexanucleotide repeat expansions in the non-coding region of the C9ORF72 gene that are translated into five dipeptide repeat (DPR) Lip Balms proteins.Here, the authors show that proline/arginine (PR) DPRs inhibit the prolyl isomerase PPIA and reveal the molecular mechanism of the impaired protein folding activity of PPIA by performing NMR measurements and determining a PR Conditioner DPR bound PPIA crystal structure.