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ProteomicsHRD1 IP-MS in WT and SEL1L deficiency HEK293T cells
To gain further insights into the role and importance of SEL1L in HRD1 ERAD, we performed unbiased proteomics LC-MS analyses to map SEL1L and HRD1 interactomes in WT and SEL1L-/-, WT and HRD1-/- HEK293T cells, respectively.
ORGANISM(S): Homo sapiens (Human) 
2024-01-10 | PXD043674 | Pride
Erad complex formation Sel1l-hrd1 erad Hek293t 
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 
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 
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 
ProteomicsHepatic Sel1L-Hrd1 ER-associated degradation deficiency leads to intrahepatic cholestasis in mice
The liver is the central organ critically regulating the balance of the metabolically potent yet toxic bile acids in the body. While genomic association studies have pointed to hepatic Sel1L – a critical component of mammalian Hrd1 ER-associated degradation (ERAD) machinery – as an influencer of ser...
ORGANISM(S): Mus musculus (Mouse) 
2023-03-11 | PXD035243 | Pride
Bile Liver Cholestasis Progressive familial intrahepatic cholestasis Abcb4 Sel1l-hrd1 erad 
GenomicsTranscriptomic responses to SEL1L-HRD1 ERAD deficiency in HEK293T cells and mouse brown adipose tissue
Transcriptomic responses to SEL1L-HRD1 ERAD deficiency in HEK293T cells and mouse brown adipose tissue
PRJNA966952 | ENA
TranscriptomicsThe ER-Associated Degradation Adapter Protein Sel1L Regulates Triglyceride Metabolism via Lipoprotein Lipase
Sel1L is an adaptor protein for the E3 ligase Hrd1 in the endoplasmic reticulum-associated degradation (ERAD), but its physiological role in a cell-type-specific manner remains unclear. Here we show that mice with adipocyte-specific Sel1L deficiency are resistant to diet-induced obesity and exhibit ...
ORGANISM(S): Mus musculus 
2015-05-18 | E-GEOD-56918 | biostudies-arrayexpress
TranscriptomicsNeuronal SEL1L-HRD1 ERAD regulates one-carbon metabolism and is essential for motor function and survival
SEL1L-HRD1 endoplasmic reticulum-associated degradation (ERAD) is essential for protein folding quality control and maintaining homeostasis in the ER. Defects in SEL1L-HRD1 ERAD complex has been linked to early onset of severe neurological syndromes. However, its physiological importance and underly...
ORGANISM(S): Mus musculus 
2026-02-10 | GSE299894 | GEO
GenomicsRegulation of endoplasmic reticulum-mitochondria contacts and mitochondrial dynamics by Sel1L-Hrd1 ERAD during thermogenesis
Regulation of endoplasmic reticulum-mitochondria contacts and mitochondrial dynamics by Sel1L-Hrd1 ERAD during thermogenesis
PRJNA608688 | ENA
GenomicsNeuronal SEL1L-HRD1 ERAD regulates one-carbon metabolism and is essential for motor function and survival
Neuronal SEL1L-HRD1 ERAD regulates one-carbon metabolism and is essential for motor function and survival
PRJNA1277270 | ENA
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