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Study shows the mechanism how loss of de-N-glycosylation enzyme causes ill effect – EurekAlert

Loss-of-mutations in NGLY1, an enzyme that removes sugar chains, cause a multisystem developmental disorder called NGLY1 deficiency….

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IMAGE: A schematic model of the mechanism of proteasome dysfunction by FBS2 in NGLY1 deficiency.
FBS2 induces cellular toxicity in the absence of NGLY by two steps. First, FBS2 ubiquitinates misfolded glycoprotein…
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Credit: TMIMS
Peptide: N-glycanase…

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