{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:38:23Z","timestamp":1791297503786,"version":"4.1.0"},"reference-count":53,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2023,8,9]],"date-time":"2023-08-09T00:00:00Z","timestamp":1691539200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005155","name":"Azrieli Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005155","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Aging Cell"],"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Autophagy is an intracellular degradative process with an important role in cellular homeostasis. Here, we show that the RNA binding protein (RBP), heterogeneous nuclear ribonucleoprotein Q (HNRNPQ)\/SYNCRIP is required to stimulate early events in autophagosome biogenesis, in particular the induction of VPS34 kinase by ULK1\u2010mediated beclin 1 phosphorylation. The RBPs HNRNPQ and poly(A) binding protein nuclear 1 (PABPN1) form a regulatory network that controls the turnover of distinct autophagy\u2010related (ATG) proteins. We also show that oculopharyngeal muscular dystrophy (OPMD) mutations engender a switch from autophagosome stimulation to autophagosome inhibition by impairing PABPN1 and HNRNPQ control of the level of ULK1. The overexpression of HNRNPQ in OPMD patient\u2010derived cells rescues the defective autophagy in these cells. Our data reveal a regulatory mechanism of autophagy induction that is compromised by PABPN1 disease mutations, and may thus further contribute to their deleterious effects.<\/jats:p>","DOI":"10.1111\/acel.13949","type":"journal-article","created":{"date-parts":[[2023,8,10]],"date-time":"2023-08-10T05:15:46Z","timestamp":1691644546000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Oculopharyngeal muscular dystrophy mutations link the <scp>RNA<\/scp>\u2010binding protein <scp>HNRNPQ<\/scp> to autophagosome biogenesis"],"prefix":"10.1111","volume":"22","author":[{"given":"Hasan","family":"Ishtayeh","sequence":"first","affiliation":[{"name":"The Department of Cell and Developmental Biology, Faculty of Medicine Tel Aviv University  Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margarita","family":"Galves","sequence":"additional","affiliation":[{"name":"The Department of Cell and Developmental Biology, Faculty of Medicine Tel Aviv University  Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tania T.","family":"Barnatan","sequence":"additional","affiliation":[{"name":"The Department of Cell and Developmental Biology, Faculty of Medicine Tel Aviv University  Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yevgeny","family":"Berdichevsky","sequence":"additional","affiliation":[{"name":"The Department of Cell and Developmental Biology, Faculty of Medicine Tel Aviv University  Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fatima","family":"Amer\u2010Sarsour","sequence":"additional","affiliation":[{"name":"The Department of Cell and Developmental Biology, Faculty of Medicine Tel Aviv University  Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Metsada","family":"Pasmanik\u2010Chor","sequence":"additional","affiliation":[{"name":"Bioinformatics Unit, G.S. Wise Faculty of Life Science Tel Aviv University  Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Itzhak","family":"Braverman","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology, Head and Neck Surgery Hillel Yaffe Medical Center  Hadera Israel"},{"name":"Rappaport Faculty of Medicine, Technion  Haifa Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sergiu C.","family":"Blumen","sequence":"additional","affiliation":[{"name":"Rappaport Faculty of Medicine, Technion  Haifa Israel"},{"name":"Department of Neurology Hillel Yaffe Medical Center  Hadera Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1253-497X","authenticated-orcid":false,"given":"Avraham","family":"Ashkenazi","sequence":"additional","affiliation":[{"name":"The Department of Cell and Developmental Biology, Faculty of Medicine Tel Aviv University  Tel Aviv Israel"},{"name":"Sagol School of Neuroscience Tel Aviv University  Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,8,9]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.4161\/auto.6.6.12376"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1080\/15548627.2016.1226733"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature22078"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.RA118.007287"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-biochem-060815-014556"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181b388a3"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/0960-8966(93)90119-5"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncb2788"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0298-164"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.hmg.a018924"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a008813"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.biocel.2006.01.016"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41580-018-0003-4"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/bts635"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2014.05.021"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100002675"},{"key":"e_1_2_10_18_1","article-title":"Transcriptional profiling of the response to starvation and fattening reveals differential regulation of autophagy genes in mammals","volume":"290","author":"Galves M.","year":"2023","journal-title":"Proceedings of the Biological Sciences"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkx1240"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-016-1683-5"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200712064"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tcb.2016.11.011"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/124.3.522"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejcb.2015.11.004"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.e08-01-0080"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2012.03.022"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/19.21.5720"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1038\/nprot.2006.47"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.4161\/auto.4451"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yexcr.2008.02.005"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1080\/15548627.2015.1100356"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tcb.2016.03.006"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature03029"},{"key":"e_1_2_10_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2018.09.048"},{"key":"e_1_2_10_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2015.11.001"},{"key":"e_1_2_10_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2011.08.037"},{"key":"e_1_2_10_37_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13059-014-0550-8"},{"key":"e_1_2_10_38_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-cellbio-092910-154005"},{"key":"e_1_2_10_39_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.201605089"},{"key":"e_1_2_10_40_1","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.258997"},{"key":"e_1_2_10_41_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.A114.627778"},{"key":"e_1_2_10_42_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/11.9.1107"},{"key":"e_1_2_10_43_1","doi-asserted-by":"publisher","DOI":"10.18632\/oncotarget.20640"},{"key":"e_1_2_10_44_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000003554"},{"key":"e_1_2_10_45_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-004-1235-2"},{"key":"e_1_2_10_46_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncb2757"},{"key":"e_1_2_10_47_1","doi-asserted-by":"publisher","DOI":"10.1111\/jnc.14638"},{"key":"e_1_2_10_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(18)30238-2"},{"key":"e_1_2_10_49_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkac194"},{"key":"e_1_2_10_50_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0810452105"},{"key":"e_1_2_10_51_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2019.11.012"},{"key":"e_1_2_10_52_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddx206"},{"key":"e_1_2_10_53_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-022-31905-0"},{"key":"e_1_2_10_54_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkl396"}],"container-title":["Aging Cell"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/acel.13949","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,16]],"date-time":"2023-10-16T07:25:44Z","timestamp":1697441144000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/acel.13949"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,8,9]]},"references-count":53,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2023,10]]}},"alternative-id":["10.1111\/acel.13949"],"URL":"https:\/\/doi.org\/10.1111\/acel.13949","archive":["Portico"],"relation":{},"ISSN":["1474-9718","1474-9726"],"issn-type":[{"value":"1474-9718","type":"print"},{"value":"1474-9726","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,8,9]]},"assertion":[{"value":"2023-02-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-07-15","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-08-09","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e13949"}}