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

T Suzuki, C Huang, H Fujihira - Gene, 2016 - Elsevier
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 …

Inhibition of NGLY1 inactivates the transcription factor Nrf1 and potentiates proteasome inhibitor cytotoxicity

FM Tomlin, UIM Gerling-Driessen, YC Liu… - ACS central …, 2017 - ACS Publications
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 …

The cytoplasmic peptide:N-glycanase (Ngly1)—basic science encounters a human genetic disorder

T Suzuki - The journal of biochemistry, 2015 - academic.oup.com
Abstract Peptide: N-glycanase (PNGase) is a de-N-glycosylating enzyme that cleaves intact
N-glycans from glycoproteins/glycopeptides. The activity of the cytoplasmic PNGase in …

Mutations in NGLY1 cause an inherited disorder of the endoplasmic reticulum–associated degradation pathway

GM Enns, V Shashi, M Bainbridge, MJ Gambello… - Genetics in …, 2014 - nature.com
Purpose: The endoplasmic reticulum–associated degradation pathway is responsible for the
translocation of misfolded proteins across the endoplasmic reticulum membrane into the …

Molecular basis and regulation of OTULIN-LUBAC interaction

PR Elliott, SV Nielsen, P Marco-Casanova, BK Fiil… - Molecular cell, 2014 - cell.com
The linear ubiquitin (Ub) chain assembly complex (LUBAC) generates Met1-linked" linear"
Ub chains that regulate the activation of the nuclear factor κB (NFκB) transcription factor and …

The UBX domain in UBXD1 organizes ubiquitin binding at the C-terminus of the VCP/p97 AAA-ATPase

M Blueggel, A Kroening, M Kracht… - Nature …, 2023 - nature.com
Abstract The AAA+ ATPase p97/VCP together with different sets of substrate-delivery
adapters and accessory cofactor proteins unfolds ubiquitinated substrates to facilitate …

Multi‐systemic involvement in NGLY1‐related disorder caused by two novel mutations

J Heeley, M Shinawi - … journal of medical genetics Part A, 2015 - Wiley Online Library
NGLY1‐related disorder is a newly described autosomal recessive condition characterized
by neurological, hepatic, ophthalmological findings and associated with dysmorphic …

Reciprocal interplay between OTULIN–LUBAC determines genotoxic and inflammatory NF-κB signal responses

M Li, L Li, S Asemota, D Kakhniashvili… - Proceedings of the …, 2022 - National Acad Sciences
Targeting nuclear factor-kappa B (NF-κB) represents a highly viable strategy against
chemoresistance in cancers as well as cell death. Ubiquitination, including linear …

[HTML][HTML] The moonlighting of RAD23 in DNA repair and protein degradation

M Grønbæk-Thygesen, C Kampmeyer… - … et Biophysica Acta (BBA …, 2023 - Elsevier
A moonlighting protein is one, which carries out multiple, often wholly unrelated, functions.
The RAD23 protein is a fascinating example of this, where the same polypeptide and the …

Newly developed Laboratory-based Size exclusion chromatography Small-angle x-ray scattering System (La-SSS)

R Inoue, T Nakagawa, K Morishima, N Sato, A Okuda… - Scientific reports, 2019 - nature.com
To understand a biological system, it is important to observe structures of biomolecules in
the solution where the system is functionalized. S mall-A ngle X-ray S cattering coupled with …