Recombinant Human Peptide-N (4)- (N-acetyl-beta-glucosaminyl)asparagine amidase (NGLY1)

Uniprot Id

Q96IV0

Target Species

Human

Target Name

NGLY1

Target Full Name

Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase

Target Function

Specifically deglycosylates the denatured form of N-linked glycoproteins in the cytoplasm and assists their proteasome-mediated degradation. Cleaves the beta-aspartyl-glucosamine (GlcNAc) of the glycan and the amide side chain of Asn, converting Asn to Asp. Prefers proteins containing high-mannose over those bearing complex type oligosaccharides. Can recognize misfolded proteins in the endoplasmic reticulum that are exported to the cytosol to be destroyed and deglycosylate them, while it has no activity toward native proteins. Deglycosylation is a prerequisite for subsequent proteasome-mediated degradation of some, but not all, misfolded glycoproteins

Target Involvement

Congenital disorder of deglycosylation (CDDG)

Target Subcellular Location

Cytoplasm.

Target Protein Families

Transglutaminase-like superfamily, PNGase family

Target Synonyms

NGLY1; PNG1; Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase; PNGase; hPNGase; EC 3.5.1.52; N-glycanase 1; Peptide:N-glycanase

Target Background

This gene encodes an enzyme that catalyzes hydrolysis of an N(4)-(acetyl-beta-D-glucosaminyl) asparagine residue to N-acetyl-beta-D-glucosaminylamine and a peptide containing an aspartate residue. The encoded enzyme may play a role in the proteasome-mediated degradation of misfolded glycoproteins. Multiple transcript variants encoding different isoforms have been found for this gene.