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TranscriptomicsTranscriptome profiling of NGLY1 deficient K562 cells
The data includes a transcriptome analysis of K562 cell lines in which the gene N-glycanase 1 (NGLY1) was mutated in exon 1 and/or exon 3 to include loss of function mutations as described in Mueller and Jakob et al, 2020. The data were used in conjunction with whole proteome MS/MS experiments to sh...
ORGANISM(S): Homo sapiens 
2020-04-20 | E-MTAB-8876 | biostudies-arrayexpress
ProteomicsComparative Proteomics of HepG2 Cells Reveals NGLY1 as an Important Regulator of Ferroptosis Resistance and Iron Uptake
NGLY1 deficiency is a rare genetic disorder caused by mutations in the NGLY1 gene. This disorder presents a wide range of clinical symptoms, and its severity varies among affected individuals. Previous studies have focused on understanding the influence of NGLY1 on energy metabolism, revealing dysre...
ORGANISM(S): Homo sapiens (Human) 
2025-07-18 | PXD058551 | Pride
Ferroptosis Hepg2 cells Ngly1 deficiency Iron metabolism 
GenomicsNGLY1-deficient cerebral organiods display abnormal neuronal differentiation
NGLY1-deficient cerebral organiods display abnormal neuronal differentiation
PRJNA595973 | ENA
GenomicsNGLY1 mutations cause protein aggregation in human neurons
NGLY1 mutations cause protein aggregation in human neurons
PRJNA810420 | ENA
GenomicsTranscriptome profiling of NGLY1 deficient K562 cells
Transcriptome profiling of NGLY1 deficient K562 cells
PRJEB37082 | ENA
GenomicsGeneration and characterization of NGLY1 patient-derived midbrain organoids
Generation and characterization of NGLY1 patient-derived midbrain organoids
PRJNA930476 | ENA
ProteomicsNGLY1 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 disability, neuropathy and seizures. NGLY1’s activity in human neural cells is currently not well understood. To understand h...
ORGANISM(S): Homo sapiens (Human) 
2024-05-23 | PXD031091 | Pride
Forebrain organoids Fragmented mitochondria Heat shock proteins Protein aggregates Ngly1 deficiency Neural cells 
GenomicsExpression signatures of NGLY1 deficiency
N-Glycanase 1 (NGLY1) deficiency is a rare and complex genetic disorder. Although recent studies have shed light on the molecular underpinnings of NGLY1 deficiency, a systematic characterization of gene and protein expression changes in patient-derived cells has been lacking. Here, we performed RNA-...
EGAS00001005573 | EGA
GenomicsRNA-seq of NGLY1-deficient patient-derived iPSC glutamatergic neurons
RNA-seq of NGLY1-deficient patient-derived iPSC glutamatergic neurons
PRJNA1463270 | ENA
ProteomicsAutophagosome Proteomics of HEK293 cells
Pan-caspase inhibitor Z-VAD-fmk acts as an inhibitor of peptide:N-glycanase (NGLY1); an endoglycosidase which cleaves N-linked glycans from glycoproteins exported from the endoplasmic reticulum during ER-associated degradation (ERAD). Pharmacological N-glycanase inhibition by Z-VAD-fmk or siRNA knoc...
ORGANISM(S): Homo sapiens (Human) 
2022-01-12 | PXD020367 | Pride
Autophagy  autophagosome  ngly1  n-glycanase  z-vad-fmk  off-target 
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