Results 1 to 10 of about 17,002 (210)

Regulation of Endoplasmic Reticulum-Associated Protein Degradation (ERAD) by Ubiquitin [PDF]

Cells, 2014
Quality control of protein folding inside the endoplasmic reticulum (ER) includes chaperone-mediated assistance in folding and the selective targeting of terminally misfolded species to a pathway called ER-associated protein degradation, or simply ERAD ...
Leticia Lemus, Veit Goder
exaly   +4 more sources

Ubiquitin-specific protease 25 functions in Endoplasmic Reticulum-associated degradation. [PDF]

PLoS ONE, 2012
Endoplasmic Reticulum (ER)-associated degradation (ERAD) discards abnormal proteins synthesized in the ER. Through coordinated actions of ERAD components, misfolded/anomalous proteins are recognized, ubiquitinated, extracted from the ER and ultimately ...
Jessica R Blount, Aaron A Burr, Amanda Denuc, Gemma Marfany, Sokol V Todi   +4 more
doaj   +3 more sources

Small molecule screening identifies cytotoxic endoplasmic reticulum-associated degradation inhibitors in multiple myeloma [PDF]

Cell Death and Disease
Multiple myeloma (MM) is an incurable plasma cell neoplasm that is highly reliant on endoplasmic reticulum-associated degradation (ERAD) to maintain protein homeostasis.
Erin M. Kropp, Sho Matono, Olivia Y. Wang, Aaron M. Robida, Malathi Kandarpa, Jineigh L. Grant, Bryndon J. Oleson, Andrew Alt, Moshe Talpaz, Matthew J. Pianko, Qing Li   +10 more
doaj   +2 more sources

Signal peptide peptidase-like proteases OsSPPL1 and OsSPPL2 facilitate ER-associated protein degradation in rice [PDF]

Nature Communications
Signal peptide peptidases (SPPs) play a critical role in intramembrane proteolysis of signal peptides in mammals. However, their function in plants remains poorly understood.
Hai-Ping Lu, Jian-Hang Xu, Jia-Xin Chang, Hui-Dan Luo, Jian-Xiang Liu   +4 more
doaj   +2 more sources

The role of ER-associated degradation and ER-phagy in health and disease [PDF]

Signal Transduction and Targeted Therapy
The endoplasmic reticulum (ER) is a major cellular organelle for the synthesis and folding of secretory and transmembrane proteins, whose proper function underpins organellar homeostasis, proper tissue function, and organismal physiology. Protein quality
Young Joo Jeon, Ze’ev A. Ronai
doaj   +2 more sources

Functional rescue of a disease-linked ERAD pathway mutation via alternative splicing [PDF]

The EMBO Journal
ER-associated degradation (ERAD) targets misfolded proteins in the endoplasmic reticulum (ER) for proteasomal degradation. Mutations in its most conserved branch involving the SEL1L-HRD1 complex cause ERAD-associated neurodevelopmental disorders with ...
Huilun Helen Wang, Zhihong Wang, Liangguang Leo Lin, Sunil K Verma, Weronika Gniadzik, Hui Wang, Zexin Jason Li, Emily Whitestone, Lulu Jiang, Muge N Kuyumcu-Martinez, Shengyi Sun, Ling Qi   +11 more
doaj   +2 more sources

Structural basis and pathological implications of the dimeric OS9-SEL1L-HRD1 ERAD Core Complex [PDF]

Nature Communications
The SEL1L-HRD1 complex represents the most conserved branch of endoplasmic reticulum (ER)-associated degradation (ERAD), a critical quality-control pathway that clears misfolded ER proteins.
Liangguang Leo Lin, Emir Maldosevic, Linyao Elina Zhou, Ahmad Jomaa, Ling Qi   +4 more
doaj   +2 more sources

Neuronal SEL1L-HRD1 ER-associated degradation is essential for motor function and survival in mice [PDF]

The Journal of Clinical Investigation
Hypomorphic variants in the SEL1L-HRD1 ER-associated degradation (ERAD) complex have been linked to severe neurological syndromes in children, including neurodevelopmental delay, intellectual disability, motor dysfunction, and early death.
Mauricio Torres, You Lu, Brent Pederson, Hui Wang, Anna Gretzinger, Liangguang L. Lin, Jiwon Hwang, Xinxin Chen, Alan C. Rupp, Abigail J. Tomlinson, Andrew J. Scott, Zhen Zhao, Daniel R. Wahl, Martin G. Myers Jr., Costas A. Lyssiotis, Ling Qi   +15 more
doaj   +2 more sources

Sugar coating autophagy: exploring the links between the inhibition of NGLY1 (N-glycanase 1) and autophagy induction

Autophagy Reports, 2023
The cytosolic enzyme NGLY1 (N-glycanase 1) is a central mediator of glycoprotein catabolism. The enzyme acts to cleave N-linked glycans from modified substrate asparagine residues prior to degradation of misfolded proteins by the proteasome, playing a ...
Holger B. R. Kramer, Sarah Ann Allman
doaj   +1 more source

Characterization of endoplasmic reticulum-associated degradation in the human fungal pathogen Candida albicans [PDF]

PeerJ, 2023
Background Candida albicans is the most prevalent human fungal pathogen. In immunocompromised individuals, C. albicans can cause serious systemic disease, and patients infected with drug-resistant isolates have few treatment options.
Ellen M. Doss, Joshua M. Moore, Bryce H. Harman, Emma H. Doud, Eric M. Rubenstein, Douglas A. Bernstein   +5 more
doaj   +2 more sources