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Cytosolic Peptide: N-Glycanase (NGLY1)-from Basic Biology to Genetic Disorder, NGLY1 Deficiency.

Advances in experimental medicine and biology - 1 Jan 2026

Fujihira Haruhiko, Suzuki Tadashi

Abstract excerpt

The cytosolic peptide:N-glycanase (PNGase, NGLY1 in mammals) is an enzyme that removes N-glycans from misfolded glycoproteins. NGLY1 contributes to cytosolic glycan degradation (non-lysosomal glycan degradation) and is one of the quality control systems for newly synthesized proteins, i.e., ER-associated degradation (ERAD). NGLY1 is also responsible for the activation of a transcription factor, NFE2L1, which...

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Identifiers

PMID: 41917400
DOI: 10.1007/978-3-032-04153-1_16

Genes

NGLY1
NFE2L1

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 8 Sept 2026.

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