N-Glycanase 1 (NGLY1)

N-glycanase is a highly conserved enzyme that catalyzes deglycosylation of misfolded N-linked glycoproteins by cleaving the glycan chain before the proteins are degraded by the proteasome.

The deduced 654-amino acid human protein contains an N-terminal PUB/PUG protein-protein interaction domain and a central transglutaminase-like motif containing the catalytic triad of the active site. Mouse and human NGLY1 share 98% identity in these 2 domains. Northern blot analysis detected Ngly1 expression in all mouse tissues examined, with highest expression in testis.In yeast, Png1 was expressed in both the cytoplasm and nucleus.