{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T03:13:47Z","timestamp":1792120427843,"version":"4.4.0"},"reference-count":35,"publisher":"Elsevier BV","issue":"15","license":[{"start":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T00:00:00Z","timestamp":1785542400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T00:00:00Z","timestamp":1785542400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2027,8,6]],"date-time":"2027-08-06T00:00:00Z","timestamp":1817510400000},"content-version":"am","delay-in-days":370,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T00:00:00Z","timestamp":1785542400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T00:00:00Z","timestamp":1785542400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T00:00:00Z","timestamp":1785542400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T00:00:00Z","timestamp":1785542400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T00:00:00Z","timestamp":1785542400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"funder":[{"DOI":"10.13039\/501100002241","name":"Japan Science and Technology Agency","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002241","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007449","name":"Takeda Science Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007449","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004398","name":"Mitsubishi Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004398","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008732","name":"The Uehara Memorial Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008732","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["cell.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Cell"],"published-print":{"date-parts":[[2026,8]]},"DOI":"10.1016\/j.molcel.2026.06.026","type":"journal-article","created":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T14:54:05Z","timestamp":1783608845000},"page":"3035-3049.e8","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":1,"title":["The UFSP2-ODR4 complex spatially confines and dynamically controls UFM1 deconjugation to safeguard neuronal proteostasis"],"prefix":"10.1016","volume":"86","author":[{"given":"Gaoxin","family":"Mao","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sota","family":"Ito","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryosuke","family":"Ishimura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun-ichi","family":"Sakamaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryohei","family":"Sasaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takefumi","family":"Uemura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kei-Ichi","family":"Ishikawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoko","family":"Komatsu-Hirota","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wado","family":"Akamatsu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manabu","family":"Abe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Waguri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sunita","family":"Bijarnia-Mahay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nobuo N.","family":"Noda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshifumi","family":"Inada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7672-7722","authenticated-orcid":false,"given":"Masaaki","family":"Komatsu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.molcel.2026.06.026_bib1","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1146\/annurev.cellbio.22.010605.093503","article-title":"Modification of proteins by ubiquitin and ubiquitin-like proteins","volume":"22","author":"Kerscher","year":"2006","journal-title":"Annu. Rev. Cell Dev. Biol."},{"key":"10.1016\/j.molcel.2026.06.026_bib2","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1021\/acs.chemrev.6b00737","article-title":"Ubiquitin-like Protein Conjugation: Structures, Chemistry, and Mechanism","volume":"118","author":"Cappadocia","year":"2018","journal-title":"Chem. Rev."},{"key":"10.1016\/j.molcel.2026.06.026_bib3","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1016\/j.molcel.2023.11.034","article-title":"The UFM1 system: Working principles, cellular functions, and pathophysiology","volume":"84","author":"Komatsu","year":"2024","journal-title":"Mol. Cell"},{"key":"10.1016\/j.molcel.2026.06.026_bib4","doi-asserted-by":"crossref","first-page":"5040","DOI":"10.1111\/febs.16730","article-title":"A guide to UFMylation, an emerging posttranslational modification","volume":"290","author":"Millrine","year":"2023","journal-title":"FEBS Journal"},{"key":"10.1016\/j.molcel.2026.06.026_bib5","doi-asserted-by":"crossref","first-page":"1977","DOI":"10.1038\/sj.emboj.7600205","article-title":"A novel protein-conjugating system for Ufm1, a ubiquitin-fold modifier","volume":"23","author":"Komatsu","year":"2004","journal-title":"EMBO J."},{"key":"10.1016\/j.molcel.2026.06.026_bib6","doi-asserted-by":"crossref","first-page":"5417","DOI":"10.1074\/jbc.M109.036814","article-title":"A novel type of E3 ligase for the Ufm1 conjugation system","volume":"285","author":"Tatsumi","year":"2010","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.molcel.2026.06.026_bib7","doi-asserted-by":"crossref","DOI":"10.15252\/embj.2022111015","article-title":"A non-canonical scaffold-type E3 ligase complex mediates protein UFMylation","volume":"41","author":"Peter","year":"2022","journal-title":"EMBO J."},{"key":"10.1016\/j.molcel.2026.06.026_bib8","doi-asserted-by":"crossref","DOI":"10.1126\/sciadv.adh3635","article-title":"Mechanistic insights into the roles of the UFM1 E3 ligase complex in ufmylation and ribosome-associated protein quality control","volume":"9","author":"Ishimura","year":"2023","journal-title":"Sci. Adv."},{"key":"10.1016\/j.molcel.2026.06.026_bib9","doi-asserted-by":"crossref","first-page":"1299","DOI":"10.1073\/pnas.1816202116","article-title":"Ribosomal protein RPL26 is the principal target of UFMylation","volume":"116","author":"Walczak","year":"2019","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.molcel.2026.06.026_bib10","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.2220340120","article-title":"RPL26\/uL24 UFMylation is essential for ribosome-associated quality control at the endoplasmic reticulum","volume":"120","author":"Scavone","year":"2023","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.molcel.2026.06.026_bib11","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1038\/s41422-019-0236-6","article-title":"UFMylation of RPL26 links translocation-associated quality control to endoplasmic reticulum protein homeostasis","volume":"30","author":"Wang","year":"2020","journal-title":"Cell Res."},{"key":"10.1016\/j.molcel.2026.06.026_bib12","article-title":"SAYSD1 senses UFMylated ribosome to safeguard co-translational protein translocation at the endoplasmic reticulum","volume":"42","author":"Wang","year":"2023","journal-title":"Cell Rep."},{"key":"10.1016\/j.molcel.2026.06.026_bib13","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1038\/s41586-024-07073-0","article-title":"UFM1 E3 ligase promotes recycling of 60S ribosomal subunits from the ER","volume":"627","author":"DaRosa","year":"2024","journal-title":"Nature"},{"key":"10.1016\/j.molcel.2026.06.026_bib14","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1038\/s41586-024-07093-w","article-title":"The UFM1 E3 ligase recognizes and releases 60S ribosomes from ER translocons","volume":"627","author":"Makhlouf","year":"2024","journal-title":"Nature"},{"key":"10.1016\/j.molcel.2026.06.026_bib15","doi-asserted-by":"crossref","DOI":"10.1126\/sciadv.adv0435","article-title":"UFMylation orchestrates spatiotemporal coordination of RQC at the ER","volume":"11","author":"Penchev","year":"2025","journal-title":"Sci. Adv."},{"key":"10.1016\/j.molcel.2026.06.026_bib16","doi-asserted-by":"crossref","first-page":"5256","DOI":"10.1074\/jbc.M610590200","article-title":"Two novel ubiquitin-fold modifier 1 (Ufm1)-specific proteases, UfSP1 and UfSP2","volume":"282","author":"Kang","year":"2007","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.molcel.2026.06.026_bib17","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1002\/1873-3468.12518","article-title":"A novel approach to assess the ubiquitin-fold modifier 1-system in cells","volume":"591","author":"Ishimura","year":"2017","journal-title":"FEBS Lett."},{"key":"10.1016\/j.molcel.2026.06.026_bib18","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1004082","article-title":"An ER complex of ODR-4 and ODR-8\/Ufm1 specific protease 2 promotes GPCR maturation by a Ufm1-independent mechanism","volume":"10","author":"Chen","year":"2014","journal-title":"PLOS Genet."},{"key":"10.1016\/j.molcel.2026.06.026_bib19","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1016\/j.bbrc.2016.05.143","article-title":"The MPN domain of Caenorhabditis elegans UfSP modulates both substrate recognition and deufmylation activity","volume":"476","author":"Ha","year":"2016","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.molcel.2026.06.026_bib20","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1038\/s41586-024-07487-w","article-title":"Accurate structure prediction of biomolecular interactions with AlphaFold 3","volume":"630","author":"Abramson","year":"2024","journal-title":"Nature"},{"key":"10.1016\/j.molcel.2026.06.026_bib21","doi-asserted-by":"crossref","DOI":"10.1038\/s41467-017-00188-1","article-title":"Ubiquitination of stalled ribosome triggers ribosome-associated quality control","volume":"8","author":"Matsuo","year":"2017","journal-title":"Nat. Commun."},{"key":"10.1016\/j.molcel.2026.06.026_bib22","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1038\/12703","article-title":"Disruption of the glucocorticoid receptor gene in the nervous system results in reduced anxiety","volume":"23","author":"Tronche","year":"1999","journal-title":"Nat. Genet."},{"key":"10.1016\/j.molcel.2026.06.026_bib23","doi-asserted-by":"crossref","first-page":"683","DOI":"10.1016\/j.ajhg.2016.06.020","article-title":"Biallelic Variants in UBA5 Link Dysfunctional UFM1 Ubiquitin-like Modifier Pathway to Severe Infantile-Onset Encephalopathy","volume":"99","author":"Muona","year":"2016","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.molcel.2026.06.026_bib24","doi-asserted-by":"crossref","first-page":"6363","DOI":"10.1007\/s12035-022-02979-0","article-title":"Deficiency of Murine UFM1-Specific E3 Ligase Causes Microcephaly and Inflammation","volume":"59","author":"Zhang","year":"2022","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.molcel.2026.06.026_bib25","doi-asserted-by":"crossref","first-page":"558","DOI":"10.7196\/SAMJnew.7917","article-title":"Identification of a mutation in the ubiquitin-fold modifier 1-specific peptidase 2 gene, UFSP2, in an extended South African family with Beukes hip dysplasia","volume":"105","author":"Watson","year":"2015","journal-title":"S. Afr. Med. J."},{"key":"10.1016\/j.molcel.2026.06.026_bib26","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1111\/cge.13134","article-title":"Novel spondyloepimetaphyseal dysplasia due to UFSP2 gene mutation","volume":"93","author":"Di Rocco","year":"2018","journal-title":"Clin. Genet."},{"key":"10.1016\/j.molcel.2026.06.026_bib27","doi-asserted-by":"crossref","first-page":"900","DOI":"10.1038\/s41436-020-01071-z","article-title":"A pathogenic UFSP2 variant in an autosomal recessive form of pediatric neurodevelopmental anomalies and epilepsy","volume":"23","author":"Ni","year":"2021","journal-title":"Genet. Med."},{"key":"10.1016\/j.molcel.2026.06.026_bib28","doi-asserted-by":"crossref","first-page":"1934","DOI":"10.1093\/brain\/awy135","article-title":"Biallelic UFM1 and UFC1 mutations expand the essential role of ufmylation in brain development","volume":"141","author":"Nahorski","year":"2018","journal-title":"Brain"},{"key":"10.1016\/j.molcel.2026.06.026_bib34","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1038\/nmeth.2019","article-title":"Fiji: an open-source platform for biological-image analysis","volume":"9","author":"Schindelin","year":"2012","journal-title":"Nat. Methods"},{"key":"10.1016\/j.molcel.2026.06.026_bib29","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/j.neures.2007.01.004","article-title":"Conditional gene targeting on the pure C57BL\/6 genetic background","volume":"58","author":"Mishina","year":"2007","journal-title":"Neurosci. Res."},{"key":"10.1016\/j.molcel.2026.06.026_bib30","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1038\/s41592-022-01488-1","article-title":"ColabFold: making protein folding accessible to all","volume":"19","author":"Mirdita","year":"2022","journal-title":"Nat. Methods"},{"key":"10.1016\/j.molcel.2026.06.026_bib31","article-title":"Generation of a control iPS cell line (JUCGRMi005-A) with no abnormalities in Parkinson's disease-related genes","volume":"74","author":"Ishikawa","year":"2024","journal-title":"Stem Cell Res."},{"key":"10.1016\/j.molcel.2026.06.026_bib32","doi-asserted-by":"crossref","first-page":"2304","DOI":"10.1016\/j.celrep.2017.10.109","article-title":"iPSC-Based Compound Screening and In Vitro Trials Identify a Synergistic Anti-amyloid beta Combination for Alzheimer's Disease","volume":"21","author":"Kondo","year":"2017","journal-title":"Cell Rep."},{"key":"10.1016\/j.molcel.2026.06.026_bib33","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1038\/nmeth.2089","article-title":"NIH Image to ImageJ: 25 years of image analysis","volume":"9","author":"Schneider","year":"2012","journal-title":"Nat. Methods"},{"key":"10.1016\/j.molcel.2026.06.026_bib35","doi-asserted-by":"crossref","DOI":"10.1038\/s41467-022-34097-9","article-title":"A distinct mammalian disome collision interface harbors K63-linked polyubiquitination of uS10 to trigger hRQT-mediated subunit dissociation","volume":"13","author":"Narita","year":"2022","journal-title":"Nat. Commun."}],"container-title":["Molecular Cell"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1097276526004156?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1097276526004156?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T12:29:29Z","timestamp":1786451369000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1097276526004156"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8]]},"references-count":35,"journal-issue":{"issue":"15","published-print":{"date-parts":[[2026,8]]}},"alternative-id":["S1097276526004156"],"URL":"https:\/\/doi.org\/10.1016\/j.molcel.2026.06.026","relation":{},"ISSN":["1097-2765"],"issn-type":[{"value":"1097-2765","type":"print"}],"subject":[],"published":{"date-parts":[[2026,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"The UFSP2-ODR4 complex spatially confines and dynamically controls UFM1 deconjugation to safeguard neuronal proteostasis","name":"articletitle","label":"Article Title"},{"value":"Molecular Cell","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.molcel.2026.06.026","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2026 Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.","name":"copyright","label":"Copyright"}]}}