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FBXO6\/FBS2, a subunit of the SCF (SKP1\u2013CUL1\u2013F-box protein) ubiquitin ligase complex, recognizes\n                    <jats:italic>N<\/jats:italic>\n                    -glycans of cytosolic glycoproteins in the endoplasmic reticulum\u2013associated degradation (ERAD) pathway. This paper reports that high levels of ERAD glycoprotein substrates abnormally ubiquitinated by SCF\n                    <jats:sup>FBS2<\/jats:sup>\n                    in the absence of NGLY1 impair the proteasome, contributing to the pathogenesis of NGLY1 deficiency. Importantly, knockout of\n                    <jats:italic>Fbxo6\/Fbs2<\/jats:italic>\n                    rescued the lethality of NGLY1 deficiency in mice, suggesting a strategy for developing therapeutics for this intractable disease.\n                  <\/jats:p>","DOI":"10.1073\/pnas.2102902118","type":"journal-article","created":{"date-parts":[[2021,7,2]],"date-time":"2021-07-02T15:45:54Z","timestamp":1625240754000},"update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":50,"title":["Loss of peptide:\n                    <i>N<\/i>\n                    -glycanase causes proteasome dysfunction mediated by a sugar-recognizing ubiquitin ligase"],"prefix":"10.1073","volume":"118","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0629-0219","authenticated-orcid":false,"given":"Yukiko","family":"Yoshida","sequence":"first","affiliation":[{"name":"Ubiquitin Project, Tokyo Metropolitan Institute of Medical Science, Tokyo 156-8506, 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