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August 8, 2026Autophagy ReportsOpen Access

The role of N-glycans and their processing in ER-to-lysosome-associated degradation of disease-causing mutant Neuroserpin

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Authors

CHCarolin HoefnerIFIlaria FregnoMMMaurizio Molinari

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Overview

Experimental analysis demonstrates the role of N-glycan glucosylation in lysosomal clearance of mutant Neuroserpin, indicating important cellular mechanisms of protein degradation.

Key Points

  • This study aims to understand how N-glycans affect the degradation process of mutant Neuroserpin linked to a specific disease.
  • Investigated the polymerization-prone Portland variant of Neuroserpin.
  • Examined the role of lectin chaperones, ER-phagy receptors, and SNARE proteins in lysosomal clearance.
  • Focused on the impact of glucosylation on the processing of N-glycans in relation to ER-to-Lysosome-Associated Degradation.
  • Showed that persistent N-glycan glucosylation signals ER-to-Lysosome-Associated Degradation mechanisms.
  • Demonstrated that the clearance of the Portland variant depends on the LC3-dependent delivery branch of ERLAD.
  • Revealed the involvement of specific proteins, including Calnexin and Syntaxin17, in the lysosomal degradation of misfolded Neuroserpin.

Cite This Study

Hoefner et al. (2026) studied this question.

synapsesocial.com/papers/6a76dae7f12abadc79815924https://doi.org/10.1080/27694127.2026.2712797
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