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ProteomicsAn ERAD independent role for rhomboid pseudoprotease Dfm1 in mediating sphingolipid homeostasis
Nearly one-third of nascent proteins are initially targeted to the endoplasmic reticulum (ER) where they are correctly folded and assembled before being delivered to their final cellular destinations. To prevent the accumulation of misfolded membrane proteins, ER-associated-degradation (ERAD) remove...
ORGANISM(S): Saccharomyces Cerevisiae (ncbitaxon:4932) 
MSV000090333 | MassIVE
UnknownEndoplasmic Reticulum-Targeted Subunit Toxins Provide a New Approach to Rescue Misfolded Mutant Proteins and Revert Cell Models of Genetic Diseases.
Not available
S-EPMC5147855 | biostudies-literature
ProteomicsCharacterization of Endoplasmic Reticulum-Associated Degradation in the human fungal pathogen C. albicans
C. 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. The Ubiquitin-Proteasome System has not been thoroughly characterized in C. albica...
ORGANISM(S): Candida Albicans (ncbitaxon:5476) 
2023-05-03 | MSV000091843 | MassIVE
TranscriptomicsSel1L is Indispensable for Mammalian ERAD, ER Homeostasis and Survival
Sel1L is an adaptor protein for the E3 ligase Hrd1 involved in endoplasmic reticulum-associated degradation (ERAD). Its physiological importance in mammalian ERAD, however, remains to be established. Here, using the inducible Sel1L knockout mouse and cell models, we provide the first in vivo evidenc...
ORGANISM(S): Mus musculus 
2013-12-04 | E-GEOD-52929 | biostudies-arrayexpress
ProteomicsHrd1 interaction proteomics - Lipid disequilibrium disrupts ER proteostasis by impairing ERAD substrate glycan trimming and dislocation
Endoplasmic reticulum (ER)-associated degradation (ERAD) mediates the degradation of misfolded and unoligomerized proteins in the early secretory pathway. ERAD substrates are detected and delivered to membrane-embedded dislocation complexes. Following transfer into the cytosol, substrates are rapidl...
ORGANISM(S): Homo sapiens (Human) 
2017-01-03 | PXD005633 | Pride
Erad Hrd1 Triacsin c 
ProteomicsRegulation of hepatic inclusions and fibrinogen biogenesis by SEL1L-HRD1 ERAD
Impaired secretion of an essential blood coagulation factor fibrinogen leads to hepatic fibrinogen storage disease (HFSD), characterized by the presence of fibrinogen-positive inclusion bodies and hypofibrinogenemia. However, the molecular mechanisms underlying the biogenesis of fibrinogen in the en...
ORGANISM(S): Mus musculus (Mouse) 
2024-09-11 | PXD047658 | Pride
Hepatocytes Sel1l-hrd1 erad Fibrinogen Inclusion bodies 
ProteomicsProteomic screens of SEL1L-HRD1 ER-associated degradation substrates reveal its vital importance in the biogenesis of glycosylphosphatidylinositol-anchored proteins
The SEL1L-HRD1 protein complex of endoplasmic reticulum (ER)-associated degradation (ERAD) plays indispensable roles for many physiological processes in a substrate-specific manner; however, the nature of endogenous substrates remains largely elusive. Here we have developed a unique proteomics strat...
ORGANISM(S): Homo sapiens (Human) 
2023-12-17 | PXD041882 | Pride
Disease mutants Endogenous substrates Pigk Sel1l-hrd1 erad Proteomic screens Gpi-anchored proteins 
ProteomicsA small molecule VDAC ligand inhibits ERAD and induces selective cancer cell death via disruption of calcium homeostasis
Endoplasmic reticulum-associated degradation (ERAD) is a critical protein quality control mechanism that also regulates lipid metabolism and calcium homeostasis. Dysregulation of ERAD and unfolded protein response underlies diseases including cancer, neurodegenerative disorders, and metabolic syndro...
ORGANISM(S): Homo sapiens (Human) 
2026-01-20 | PXD071546 | Pride
Erad Proteomics Ingel digestion Er-phagy Vdac 
TranscriptomicsTranscription profiling of human cell cancer line treated with bortezomib or Eeyarestatin I (EerI) reveals ERAD inhibitors integrate ER stress with an epigenetic mechanism to activate BH3 only protein NOXA in cancer cells
The ubiquitin-proteasome system (UPS) has recently emerged as a major target for drug development in cancer therapy. The proteasome inhibitor bortezomib has clinical activity in multiple myeloma and mantle cell lymphoma. Here we report that Eeyarestatin I (EerI), a chemical inhibitor that blocks ER-...
ORGANISM(S): Homo sapiens 
2009-02-28 | E-GEOD-14003 | biostudies-arrayexpress
ProteomicsDirect visualization of ERAD nanocompartments within the cell
ER-associated degradation (ERAD) is an evolutionarily-conserved quality control mechanism where misfolded proteins are removed from the endoplasmic reticulum (ER) and degraded by the ubiquitin-proteasome system. While many ERAD-mediating proteins have been identified, it is unknown how they are spat...
ORGANISM(S): Chlamydomonas reinhardtii 
2020-05-26 | PXD009375 | Pride
Erad electron microscopy 
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