NGLY1 Antibodies, cDNA Clones Research Reagents

NGLY1 (N-Glycanase 1, also known as CDDG; PNG1; CDG1V; PNGase), located on 3p24.2, is a Protein Coding gene. 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 gene produces a 74390 Da protein composed of 654 amino acids. This enzyme is known to be involved in the quality control system for the newly synthesized glycoproteins in the endoplasmic reticulum (ER). Diseases such as Congenital Disorder Of Deglycosylation and Peripheral Nervous System Disease are associated with NGLY1.
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NGLY1 General Information

NGLY1 Antibody (1)

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    NGLY1 cDNA Clone (15)

    NGLY1 qPCR Primer (1)

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    NGLY1 Background

    NGLY1 deficiency is a rare inherited disorder caused by mutations in the NGLY1 gene encoding N-glycanase 1 that is a hydrolase for N-linked glycosylated proteins. NGLY1/Ngly1 is a cytosolic peptide: N-glycanase, i.e. de-N-glycosylating enzyme acting on N-glycoproteins in mammals, generating free, unconjugated N-glycans and deglycosylated peptides in which the N-glycosylated asparagine residues are converted to aspartates. The cytosolic enzyme N-glycanase 1, encoded by NGLY1, catalyzes the cleavage of the β-aspartyl glycosylamine bond of N-linked glycoproteins, releasing intact N-glycans from proteins bound for degradation. This enzyme is known to be involved in the quality control system for the newly synthesized glycoproteins in the endoplasmic reticulum (ER). In this system, misfolded (Glyco)proteins are retro-translocated to the cytosol, where the 26S proteasomes play a central role in degrading the proteins: a process referred to as ER-associated degradation or ERAD in short.

    NGLY1 References

    • Yang K, et al. (2018) N-glycanase ngly1 regulates mitochondrial homeostasis and inflammation through nrf1. J Exp Med 215 (10): 2600-2616.
    • Suzuki T, et al. (2016) The cytoplasmic peptide:N-glycanase (ngly1) - structure, expression and cellular functions. Gene 577 (1): 1-7.
    • Lam C, et al. (2017) Prospective phenotyping of ngly1-cddg, the first congenital disorder of deglycosylation. Genet Med 19 (2): 160-168.

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