The NGLY1 gene provides instructions for making an enzyme called N-glycanase 1. This enzyme is involved in a process called deglycosylation, by which chains of sugar molecules (glycans) are removed from proteins. Specifically, N-glycanase 1 removes glycans from misfolded proteins. This step is thought to be essential for certain abnormal proteins to be broken down (degraded).
Other Names
CDDG; CDGIV; FLJ11005; hPNGase; peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase isoform 1; peptide:N-glycanase; PNG1; PNGase
Related Genetics Topics
Identifiers
- NCBI Gene: 55768
- OMIM: 610661
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
