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ProteomicsCoalescence of endoplasmic reticulum fragments form a new quality-control compartment in adipocytes
Using a combination of state-of-the-art (ultra-) imaging, biochemical and biophysical techniques, this study not only uncover a new cellular quality-control compartment, but demonstrate a profound cellular adaptation capability and plasticity in dealing with misfolded proteins or protein aggregates ...
ORGANISM(S): Mus musculus (Mouse) 
2023-04-25 | PXD038310 | Pride
Autophagy Lipoprotein lipase Tryptophan loop Sel1l-hrd1 erad Cerfs Bip Er-phagy Protein aggregation Phase separation Soluble-to-insoluble phase transition 
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 
ProteomicsThe mechanism to dispose of misfolded proteins in the endoplasmic reticulum of adipocytes
Endoplasmic reticulum (ER)-associated degradation (ERAD) and ER-phagy are two principal degradative mechanisms in the ER; however, the crosstalk between these two pathways and its physiological significance remain unexplored. Here we report that SEL1L-HRD1 ERAD limits autophagy and that, when ERAD i...
ORGANISM(S): Mus musculus (Mouse) 
2023-04-25 | PXD040899 | Pride
Autophagy Lipoprotein lipase Tryptophan loop Sel1l-hrd1 erad Cerfs Bip Er-phagy Protein aggregation Phase separation Soluble-to-insoluble phase transition 
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) Mus musculus (Mouse) 
2023-12-17 | PXD041803 | Pride
Endogenous substrates Pigk Disease mutants   Sel1l-hrd1 erad Proteomic screens Gpi-anchored proteins 
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 
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