NGLY1 : N-glycanase 1

Description

The NGLY1 (N-glycanase 1) is a protein-coding gene located on chromosome 3.

The NGLY1 gene provides instructions for making an enzyme called N-glycanase 1. This enzyme is involved in removing chains of sugar molecules (glycans) from proteins, a process called deglycosylation. Specifically, N-glycanase 1 removes glycans from misfolded proteins, which is thought to be essential for the breakdown (degradation) of certain abnormal proteins.

NGLY1 is also known as CDDG, CDG1V, PNG-1, PNG1, PNGase.

Associated Diseases

• Alacrimia-choreoathetosis-liver dysfunction syndrome
• Congenital disorder of deglycosylation 1
• NGLY1-congenital disorder of deglycosylation