Endo-β-N-acetylglucosaminidase forms N-GlcNAc protein aggregates during ER-associated degradation in Ngly1-defective cells
about
The cytoplasmic peptide:N-glycanase (NGLY1) - Structure, expression and cellular functionsLethality of mice bearing a knockout of the Ngly1-gene is partially rescued by the additional deletion of the Engase gene.Forcible destruction of severely misfolded mammalian glycoproteins by the non-glycoprotein ERAD pathwayGeneration and degradation of free asparagine-linked glycans.Non-lysosomal Degradation of Singly Phosphorylated Oligosaccharides Initiated by the Action of a Cytosolic Endo-β-N-acetylglucosaminidaseProspective phenotyping of NGLY1-CDDG, the first congenital disorder of deglycosylation.Tissue-specific regulation of BMP signaling by Drosophila N-glycanase 1.Generation of a Mutant Mucor hiemalis Endoglycosidase That Acts on Core-fucosylated N-Glycans.Inhibition of NGLY1 Inactivates the Transcription Factor Nrf1 and Potentiates Proteasome Inhibitor Cytotoxicity.Cytosolic-free oligosaccharides are predominantly generated by the degradation of dolichol-linked oligosaccharides in mammalian cells.Mitochondrial function requires NGLY1.Occurrence of free sialyl oligosaccharides related to N-glycans (sialyl free N-glycans) in animal sera.Repurposing of Proton Pump Inhibitors as first identified small molecule inhibitors of endo-β-N-acetylglucosaminidase (ENGase) for the treatment of NGLY1 deficiency, a rare genetic disease.Transcriptome and functional analysis in a Drosophila model of NGLY1 deficiency provides insight into therapeutic approaches.Dissecting PUGNAc-mediated inhibition of the pro-survival action of insulin.Defects in the Neuroendocrine Axis Contribute to Global Development Delay in a Drosophila Model of NGLY1 Deficiency.
P2860
Q30274598-A434F082-C879-4876-B34A-8521A98DF796Q33584478-56269373-F2C9-4962-A01A-3CC232F61D1BQ34501894-B6C75075-ADF8-496D-B56F-8843625675D0Q38378683-9E98B197-650E-4CD8-A78D-9E7A315F9112Q38795510-8BECC411-3093-4407-B8A1-9F536913B10FQ39623619-6383BA8C-063B-4AB0-ABA6-4FECF270B08CQ41063018-8645B29B-8606-4D29-8F2D-67D3A6648473Q42725794-F31657B4-317A-4D67-96C9-59BDB63479C1Q45074664-1509E536-E08A-4DC9-82D9-59D5EEEC139EQ45345660-5C05C836-679E-44C2-9ADE-EBB8225AC7E3Q46329723-A23A793D-8236-45DE-8485-44222028B71AQ46560069-8F60B13E-92EE-47E8-AF1C-5007F17E8AD6Q47575481-4A0771ED-8A4A-4BE7-A0FD-3A0A5DA994A8Q49964423-1863EA08-183F-40EC-BDF1-D82AEF1881C3Q53144959-82B2A859-F17C-4595-A071-3DBD78336FD4Q55443680-82016A50-50CF-46C8-9E97-EE259AAE374E
P2860
Endo-β-N-acetylglucosaminidase forms N-GlcNAc protein aggregates during ER-associated degradation in Ngly1-defective cells
description
2015 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
2015 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
article publié dans les Procee ...... f the United States of America
@fr
artículu científicu espublizáu en 2015
@ast
im Februar 2015 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 2015/02/03)
@sk
vědecký článek publikovaný v roce 2015
@cs
wetenschappelijk artikel (gepubliceerd op 2015/02/03)
@nl
наукова стаття, опублікована в лютому 2015
@uk
name
Endo-β-N-acetylglucosaminidase ...... ation in Ngly1-defective cells
@ast
Endo-β-N-acetylglucosaminidase ...... ation in Ngly1-defective cells
@en
Endo-β-N-acetylglucosaminidase ...... ation in Ngly1-defective cells
@nl
type
label
Endo-β-N-acetylglucosaminidase ...... ation in Ngly1-defective cells
@ast
Endo-β-N-acetylglucosaminidase ...... ation in Ngly1-defective cells
@en
Endo-β-N-acetylglucosaminidase ...... ation in Ngly1-defective cells
@nl
prefLabel
Endo-β-N-acetylglucosaminidase ...... ation in Ngly1-defective cells
@ast
Endo-β-N-acetylglucosaminidase ...... ation in Ngly1-defective cells
@en
Endo-β-N-acetylglucosaminidase ...... ation in Ngly1-defective cells
@nl
P2093
P2860
P356
P1476
Endo-β-N-acetylglucosaminidase ...... ation in Ngly1-defective cells
@en
P2093
Akira Hosomi
Chengcheng Huang
Haruhiko Fujihira
Junichi Seino
Naoshi Dohmae
Tadashi Suzuki
Takehiro Suzuki
Yoichiro Harada
Yoko Funakoshi
Yuki Masahara-Negishi
P2860
P304
P356
10.1073/PNAS.1414593112
P407
P577
2015-02-03T00:00:00Z