The cytoplasmic peptide: N-glycanase (NGLY1)—Structure, expression and cellular functions
NGLY1/Ngly1 is a cytosolic peptide: N-glycanase, ie. de-N-glycosylating enzyme acting on
N-glycoproteins in mammals, generating free, unconjugated N-glycans and deglycosylated …
N-glycoproteins in mammals, generating free, unconjugated N-glycans and deglycosylated …
Evaluating the clinical validity of gene-disease associations: an evidence-based framework developed by the clinical genome resource
With advances in genomic sequencing technology, the number of reported gene-disease
relationships has rapidly expanded. However, the evidence supporting these claims varies …
relationships has rapidly expanded. However, the evidence supporting these claims varies …
NGLY1 deficiency, a congenital disorder of deglycosylation: from disease gene function to pathophysiology
N-Glycanase 1 (NGLY1) is a cytosolic enzyme involved in removing N-linked glycans of
misfolded N-glycoproteins and is considered to be a component of endoplasmic reticulum …
misfolded N-glycoproteins and is considered to be a component of endoplasmic reticulum …
Inhibition of NGLY1 inactivates the transcription factor Nrf1 and potentiates proteasome inhibitor cytotoxicity
Proteasome inhibitors are used to treat blood cancers such as multiple myeloma (MM) and
mantle cell lymphoma. The efficacy of these drugs is frequently undermined by acquired …
mantle cell lymphoma. The efficacy of these drugs is frequently undermined by acquired …
Prospective phenotyping of NGLY1-CDDG, the first congenital disorder of deglycosylation
C Lam, C Ferreira, D Krasnewich, C Toro… - Genetics in …, 2017 - nature.com
Purpose: The cytosolic enzyme N-glycanase 1, encoded by NGLY1, catalyzes cleavage of
the β-aspartyl glycosylamine bond of N-linked glycoproteins, releasing intact N-glycans from …
the β-aspartyl glycosylamine bond of N-linked glycoproteins, releasing intact N-glycans from …
[HTML][HTML] N-glycanase NGLY1 regulates mitochondrial homeostasis and inflammation through NRF1
Mutations in the NGLY1 (N-glycanase 1) gene, encoding an evolutionarily conserved
deglycosylation enzyme, are associated with a rare congenital disorder leading to global …
deglycosylation enzyme, are associated with a rare congenital disorder leading to global …
N-Glycosylation
T Hirata, Y Kizuka - The role of glycosylation in health and disease, 2021 - Springer
N-glycosylation is a highly conserved glycan modification, and more than 7000 proteins are
N-glycosylated in humans. N-glycosylation has many biological functions such as protein …
N-glycosylated in humans. N-glycosylation has many biological functions such as protein …
NGLY1: a fascinating, multifunctional molecule
T Suzuki, H Fujihira - Biochimica et Biophysica Acta (BBA)-General …, 2024 - Elsevier
NGLY1, a cytoplasmic de-N-glycosylating enzyme is well conserved among eukaryotes.
This enzyme has attracted considerable attention after mutations on the NGLY1 gene were …
This enzyme has attracted considerable attention after mutations on the NGLY1 gene were …
Lethality of mice bearing a knockout of the Ngly1-gene is partially rescued by the additional deletion of the Engase gene
H Fujihira, Y Masahara-Negishi, M Tamura… - PLoS …, 2017 - journals.plos.org
The cytoplasmic peptide: N-glycanase (Ngly1 in mammals) is a de-N-glycosylating enzyme
that is highly conserved among eukaryotes. It was recently reported that subjects harboring …
that is highly conserved among eukaryotes. It was recently reported that subjects harboring …
NGLY1 mutations cause protein aggregation in human neurons
Biallelic mutations in the gene that encodes the enzyme N-glycanase 1 (NGLY1) cause a
rare disease with multi-symptomatic features including developmental delay, intellectual …
rare disease with multi-symptomatic features including developmental delay, intellectual …