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Recognizing misfolded proteins in the endoplasmic reticulum

The mechanism by which cells recognize misfolded proteins in the endoplasmic reticulum has been defined at the cell biological level. The enzyme UDP-glucose:glycoprotein glucosyltransferase identifies and specifically glucosylates misfolded domains within a multi-domain protein.

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Figure 1: De- and re-glucosylation cycle of the quality control system in the endoplasmic reticulum (ER).

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Matouschek, A. Recognizing misfolded proteins in the endoplasmic reticulum. Nat Struct Mol Biol 7, 265–266 (2000). https://doi.org/10.1038/74012

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