Results 71 to 80 of about 933 (165)

Coupled deglycosylation-ubiquitination cascade in regulating PD-1 degradation by MDM2

Cell Reports, 2023
Summary: Posttranslational modifications represent a key step in modulating programmed death-1 (PD-1) functions, but the underlying mechanisms remain incompletely defined. Here, we report crosstalk between deglycosylation and ubiquitination in regulating
Zhen Wu, Zhijie Cao, Han Yao, Xiaojun Yan, Wenbin Xu, Mi Zhang, Zishan Jiao, Zijing Zhang, Jianyuan Chen, Yajing Liu, Meng Zhang, Donglai Wang   +11 more
doaj   +1 more source

The cytoplasmic peptide:N-glycanase (NGLY1) — Structure, expression and cellular functions [PDF]

Gene, 2016
NGLY1/Ngly1 is a cytosolic peptide:N-glycanase, i.e. de-N-glycosylating enzyme acting on N-glycoproteins in mammals, generating free, unconjugated N-glycans and deglycosylated peptides in which the N-glycosylated asparagine residues are converted to aspartates.
Suzuki, Tadashi, Huang, Chengcheng, Fujihira, Haruhiko   +2 more
openaire   +2 more sources

Expression and DNA methylation of 20S proteasome subunits as prognostic and resistance markers in cancer

Molecular Oncology, Volume 19, Issue 12, Page 3729-3749, December 2025.
Comprehensive analysis of genomic mutations, gene expression, DNA methylation, and pathway analysis of TCGA data was carried out to define cancer types in which proteasome subunits expression is associated with worse survival. Albeit the effect of specific proteasome subunits on cellular function, the main role of the proteasome is better evaluated ...
Ruba Al‐Abdulla, Simone Venz, Ruslan Al‐Ali, Martin Wendlandt, Mandy Radefeldt, Elke Krüger   +5 more
wiley   +1 more source

AAV9-mediated NGLY1 gene replacement suppresses non-epileptic convulsions in Ngly1 rats

Biochemical and Biophysical Research Communications
N-glycanase 1 (NGLY1) deficiency is a rare autosomal-recessive neurological disorder characterized by neurological dysfunction and so far, has no effective therapy. A systemic Ngly1-/- rat model recapitulates many patient symptoms, including developmental delay, motor and cognitive deficits.
Makoto Asahina, Reiko Fujinawa, Hiroto Hirayama, Hiroshi Yukitake, Tadashi Suzuki   +4 more
openaire   +2 more sources

Novel small-molecule modifiers rescue pathology associated with NGLY1 Deficiency in a Caenorhabditis elegans model

, 2021
Functional loss in clearing misfolded proteins is associated with several human diseases. A mutation in one such gene, NGLY1 results in a rare genetic disorder in humans.
Broussalian, Michael
core   +1 more source

Variants in NGLY1 lead to intellectual disability, myoclonus epilepsy, sensorimotor axonal polyneuropathy and mitochondrial dysfunction. [PDF]

, 2020
NGLY1 encodes the enzyme N-glycanase that is involved in the degradation of glycoproteins as part of the endoplasmatic reticulum-associated degradation pathway.
Brandt, F. van den, Hendriks, Yvonne, Smeitink, Jan A M, Hasselt, P.M. van, Emst-de Vries, S.E. van, Lefeber, Dirk J, Wortmann, Saskia B, de Vries, Maaike, Lefeber, Dirk J., Rodenburg, R.J., Küsters, B., de Vries, M., Rodenburg, Richard J, Wolf, N.I., van Emst-de Vries, Sjenet, Westerlo, E.M.A. van de, Vries, M.C. de, Koopman, Werner J.H., Panneman, Daan M, van Hasselt, Peter M., Wortmann, Saskia B., van den Brandt, F., van den Brandt, Frans, Smeitink, J.A.M., Rodenburg, R.J.T., Panneman, Daan M., Wortmann, S.B., Lefeber, D.J., Panneman, D.M., Wintjes, L., Haaxma, C.A., van de Westerlo, E., van Hasselt, P.M., Wintjes, Liesbeth, Koopman, Werner J H, Wolf, Nicole I., van Emst-de Vries, S., Smeitink, Jan A.M., Rodenburg, Richard J., Haaxma, Charlotte A, van de Westerlo, Els, Hendriks, Y., van Hasselt, Peter M, Wolf, Nicole I, Koopman, W.J.H., Haaxma, Charlotte A., Küsters, Benno   +46 more
core   +2 more sources

Stress and interferon signalling-mediated apoptosis contributes to pleiotropic anticancer responses induced by targeting NGLY1

, 2018
BACKGROUND: Although NGLY1 is known as a pivotal enzyme that catalyses the deglycosylation of denatured glycoproteins, information regarding the responses of human cancer and normal cells to NGLY1 suppression is limited.
Ho, Yin Ying, Kyle A. Emmitte, Sampat, R., Abhishek Parab, Emmitte, Kyle A., Victor. J. T. Lin, Emmitte, K.A., Jin Liu, Mishra, N.M., Wang, Y.C., Parab, Abhishek, Wang, Yu-Chieh, Hoffmann, P., Parab, A., Lin, Victor J. T., Liu, J., Zolekar, A., Xiaoyan Liao, Hoffmann, Peter, Ashwini Zolekar, Hamed S. Hayatshahi, Yu-Chieh Wang, Liu, Jin, Lin, V.J.T., Nigam M. Mishra, Hayatshahi, H.S., Liao, X., Peter Hoffmann, Ho, Y.Y., Liao, Xiaoyan, Zolekar, Ashwini, Hayatshahi, Hamed S., Sampat, Rohit, Rohit Sampat, Mishra, Nigam M., Yin Ying Ho   +35 more
core   +1 more source

An induced pluripotent stem cell line (TRNDi010-C) from a patient carrying a homozygous p.R401X mutation in the NGLY1 gene

Stem Cell Research, 2019
NGLY1 deficiency is a rare inherited disorder caused by mutations in the NGLY1 gene encoding N-glycanase 1 that is a hydrolase for N-linked glycosylated proteins. An induced pluripotent stem cell (iPSC) line was generated from the dermal fibroblasts of a
Shu Yang, Yu-Shan Cheng, Rong Li, Manisha Pradhan, Junjie Hong, Jeanette Beers, Jizhong Zou, Chengyu Liu, Matt Might, Steven Rodems, Wei Zheng   +10 more
doaj   +1 more source

Antigen presentation of post‐translationally modified peptides in major histocompatibility complexes

Immunology &Cell Biology, Volume 103, Issue 2, Page 161-177, February 2025.
T cells recognize pathogens and malignantly transformed cells through antigen presentation on major histocompatibility complex molecules. Post‐translational modifications (PTMs) of proteins can alter the peptides presented, influencing immune recognition and disease.
Alexine S de Wit, Frans Bianchi, Geert van den Bogaart   +2 more
wiley   +1 more source

Molecular Mechanisms of Synergistic Effect of PRIMA‐1met and Oxaliplatin in Colorectal Cancer With Different p53 Status

Cancer Medicine, Volume 14, Issue 1, January 2025.
We report differential molecular mechanisms including pathways, key modules, and hub genes, induced by combination of oxaliplatin (L‐OHP) and PRIMA‐1met (APR‐246, eprenetapopt) in p53‐wild type vs. p53‐mutant colorectal cancer. Our in vivo studies show that the additional of PRIMA‐1met offers a threefold advantage: enhanced therapeutic effects, reduced
Xiao‐lan Li, Jianbiao Zhou, Nicole Xin‐Ning Tang, Yi Chai, Meng Zhou, Ai‐di Gao, Zhong‐kai Lu, Han Min   +7 more
wiley   +1 more source