{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Li S"],"funding":["HHS | NIH | National Institute of General Medical Sciences","HHS | NIH | National Institute of Arthritis and Musculoskeletal and Skin Diseases","HHS | NIH | National Institute of Neurological Disorders and Stroke","NINDS NIH HHS","NIAMS NIH HHS","NIGMS NIH HHS"],"pagination":["25104-25115"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC7547246"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["117(40)"],"pubmed_abstract":["Maintaining the fidelity of nascent peptide chain (NP) synthesis is essential for proteome integrity and cellular health. Ribosome-associated quality control (RQC) serves to resolve stalled translation, during which untemplated Ala/Thr residues are added C terminally to stalled peptide, as shown during C-terminal Ala and Thr addition (CAT-tailing) in yeast. The mechanism and biological effects of CAT-tailing-like activity in metazoans remain unclear. Here we show that CAT-tailing-like modification of poly(GR), a dipeptide repeat derived from amyotrophic lateral sclerosis with frontotemporal dementia (ALS/FTD)-associated <i>GGGGCC</i> (G4C2) repeat expansion in <i>C9ORF72</i>, contributes to disease. We find that poly(GR) can act as a mitochondria-targeting signal, causing some poly(GR) to "],"journal":["Proceedings of the National Academy of Sciences of the United States of America"],"pubmed_title":["Quality-control mechanisms targeting translationally stalled and C-terminally extended poly(GR) associated with ALS/FTD."],"pmcid":["PMC7547246"],"funding_grant_id":["R01GM115898","R01NS083417","R01 GM115898","R01NS084412","R01 NS084412","R01AR0748750","R01 NS083417","R01 AR074875"],"pubmed_authors":["Wu Z","Vogel H","Li Y","Li S","Glynn S","Dong J","Tantray I","Snyder M","Chen S","Lu B"],"additional_accession":[]},"is_claimable":false,"name":"Quality-control mechanisms targeting translationally stalled and C-terminally extended poly(GR) associated with ALS/FTD.","description":"Maintaining the fidelity of nascent peptide chain (NP) synthesis is essential for proteome integrity and cellular health. Ribosome-associated quality control (RQC) serves to resolve stalled translation, during which untemplated Ala/Thr residues are added C terminally to stalled peptide, as shown during C-terminal Ala and Thr addition (CAT-tailing) in yeast. The mechanism and biological effects of CAT-tailing-like activity in metazoans remain unclear. Here we show that CAT-tailing-like modification of poly(GR), a dipeptide repeat derived from amyotrophic lateral sclerosis with frontotemporal dementia (ALS/FTD)-associated <i>GGGGCC</i> (G4C2) repeat expansion in <i>C9ORF72</i>, contributes to disease. We find that poly(GR) can act as a mitochondria-targeting signal, causing some poly(GR) to ","dates":{"release":"2020-01-01T00:00:00Z","publication":"2020 Oct","modification":"2025-04-04T11:53:50.062Z","creation":"2025-04-04T11:53:50.062Z"},"accession":"S-EPMC7547246","cross_references":{"pubmed":["32958650"],"doi":["10.1073/pnas.2005506117"]}}