Article
Rescue of secretion of rare-disease-associated misfolded mutant glycoproteins in UGGT1 knock-out mammalian cells.
Traffic (Copenhagen, Denmark) - 1 Jan 2024
Tax Gabor, Guay Kevin P, Pantalone Ludovica, Ceci Martina, Soldà Tatiana, Hitchman Charlie J, Hill Johan C, Vasiljević Snežana, Lia Andrea, Modenutti Carlos P, Straatman Kees R, Santino Angelo, Molinari Maurizio, Zitzmann Nicole, Hebert Daniel N, Roversi Pietro, Trerotola Marco
Abstract excerpt
Endoplasmic reticulum (ER) retention of misfolded glycoproteins is mediated by the ER-localized eukaryotic glycoprotein secretion checkpoint, UDP-glucose glycoprotein glucosyl-transferase (UGGT). The enzyme recognizes a misfolded glycoprotein and flags it for ER retention by re-glucosylating one of its N-linked glycans. In the background of a congenital mutation in a secreted glycoprotein gene, UGGT-mediated ER...
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