Background The quality of proteins destined for the secretory pathway is ensured by two
distinct mechanisms in the endoplasmic reticulum (ER): productive folding of newly …

Highlights•Neural diseases and neuropsychiatric diseases are growing global
problems.•Effective therapies for neurological diseases are still unmet medical …

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 …

Mutations in the NGLY1 (N-glycanase 1) gene, encoding an evolutionarily conserved
deglycosylation enzyme, are associated with a rare congenital disorder leading to global …

Mutations in the human peptide: N-glycanase gene (NGLY1), which encodes a cytosolic de–
N-glycosylating enzyme, cause a congenital autosomal recessive disorder. In rodents, the …

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 …

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 …

N-Glycanase 1 (NGLY1) is a cytoplasmic deglycosylating enzyme. Loss-of-function
mutations in the NGLY1 gene cause NGLY1 deficiency, which is characterized by …

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 …

During endoplasmic reticulum-associated degradation (ERAD), the cytoplasmic enzyme N-
glycanase 1 (NGLY1) is proposed to remove N-glycans from misfolded N-glycoproteins after …