D. N. Hebert

Rescue of secretion of a rare-disease associated mis-folded mutant glycoprotein in UGGT1 knock-out mammalian cells

This study shows that knocking out UGGT1 or UGGT1/2 genes rescues secretion of a misfolded Trop-2 glycoprotein mutant in mammalian cells.

The conformational landscape of a serpin N-terminal subdomain facilitates folding and in-cell quality control

This paper investigates how the N-terminal subdomain of serpin 1-antitrypsin (AAT) influences folding and in-cell quality control by maintaining a dynamic conformational landscape.

A quinolin-8-ol sub-millimolar inhibitor of UGGT, the ER glycoprotein folding quality control checkpoint

This paper identifies a small molecule inhibitor of UGGT, a key enzyme in ER glycoprotein folding, and demonstrates its inhibitory effects in vitro and in cell culture.

Quantitative glycoproteomics reveals cellular substrate selectivity of the ER protein quality control sensors UGGT1 and UGGT2

This study identifies UGGT1 and UGGT2 substrates using quantitative glycoproteomics, revealing their distinct substrate preferences and roles in ER protein quality control.

ER transmembrane protein TMTC3 contributes to O-mannosylation of E-cadherin, Cellular Adherence and Embryonic Gastrulation

This study shows TMTC3 enables O-mannosylation of E-cadherin, affecting cell adhesion and gastrulation, and links TMTC3 mutations to Cobblestone lissencephaly.