{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T11:13:55Z","timestamp":1790680435185,"version":"4.1.0"},"reference-count":149,"publisher":"The Company of Biologists","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2014,3,1]]},"abstract":"<jats:p>Biosynthesis of proteins \u2013 from translation to folding to export \u2013 encompasses a complex set of events that are exquisitely regulated and scrutinized to ensure the functional quality of the end products. Cells have evolved to capitalize on multiple post-translational modifications in addition to primary structure to indicate the folding status of nascent polypeptides to the chaperones and other proteins that assist in their folding and export. These modifications can also, in the case of irreversibly misfolded candidates, signal the need for dislocation and degradation. The current Review focuses on the glycoprotein quality-control (GQC) system that utilizes protein N-glycosylation and N-glycan trimming to direct nascent glycopolypeptides through the folding, export and dislocation pathways in the endoplasmic reticulum (ER). A diverse set of pathological conditions rooted in defective as well as over-vigilant ER quality-control systems have been identified, underlining its importance in human health and disease. We describe the GQC pathways and highlight disease and animal models that have been instrumental in clarifying our current understanding of these processes.<\/jats:p>","DOI":"10.1242\/dmm.014589","type":"journal-article","created":{"date-parts":[[2014,3,6]],"date-time":"2014-03-06T12:26:18Z","timestamp":1394108778000},"page":"331-341","source":"Crossref","is-referenced-by-count":93,"title":["Glycoprotein folding and quality-control mechanisms in protein-folding diseases"],"prefix":"10.1242","volume":"7","author":[{"given":"Sean P.","family":"Ferris","sequence":"first","affiliation":[{"name":"Department of Biological Chemistry and Medical Scientist Training Program, University of Michigan, Ann Arbor, MI 48109, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vamsi K.","family":"Kodali","sequence":"additional","affiliation":[{"name":"Center for Neuroscience, Aging and Stem Cell Research, Sanford-Burnham Medical Research Institute, 10901 N. 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