Gene Ontology annotation based on Enzyme Commission mapping
Manual transfer of experimentally-verified manual GO annotation data to orthologs by curator judgment of sequence similarity
Annotation inferences using phylogenetic trees
Gene Ontology annotation based on UniPathway vocabulary mapping
Gene Ontology annotation based on UniProtKB/Swiss-Prot keyword mapping
Gene Ontology annotation based on UniProtKB/Swiss-Prot Subcellular Location vocabulary mapping, accompanied by conservative changes to GO terms applied by UniProt
Gene Ontology annotation based on curation of immunofluorescence data
Automatic transfer of experimentally verified manual GO annotation data to orthologs using Ensembl Compara
Electronic Gene Ontology annotations created by ARBA machine learning models
Human HRD1 protects against ER stress-induced apoptosis through ER-associated degradation.
Human HRD1 is an E3 ubiquitin ligase involved in degradation of proteins from the endoplasmic reticulum.
Recruitment of the p97 ATPase and ubiquitin ligases to the site of retrotranslocation at the endoplasmic reticulum membrane.
A ubiquitin ligase HRD1 promotes the degradation of Pael receptor, a substrate of Parkin.
Cytoplasmic destruction of p53 by the endoplasmic reticulum-resident ubiquitin ligase 'Synoviolin'.
OS-9 and GRP94 deliver mutant alpha1-antitrypsin to the Hrd1-SEL1L ubiquitin ligase complex for ERAD.
Synoviolin promotes IRE1 ubiquitination and degradation in synovial fibroblasts from mice with collagen-induced arthritis.
Human XTP3-B forms an endoplasmic reticulum quality control scaffold with the HRD1-SEL1L ubiquitin ligase complex and BiP.
CYP3A4 ubiquitination by gp78 (the tumor autocrine motility factor receptor, AMFR) and CHIP E3 ligases.
USP14 inhibits ER-associated degradation via interaction with IRE1alpha.
A comprehensive framework of E2-RING E3 interactions of the human ubiquitin-proteasome system.
Defining the membrane proteome of NK cells.
Mannose trimming is required for delivery of a glycoprotein from EDEM1 to XTP3-B and to late endoplasmic reticulum-associated degradation steps.
HRD1 and UBE2J1 target misfolded MHC class I heavy chains for endoplasmic reticulum-associated degradation.
Membrane-associated ubiquitin ligase complex containing gp78 mediates sterol-accelerated degradation of 3-hydroxy-3-methylglutaryl-coenzyme A reductase.
SEL1L protein critically determines the stability of the HRD1-SEL1L endoplasmic reticulum-associated degradation (ERAD) complex to optimize the degradation kinetics of ERAD substrates.
A ubiquitin ligase-associated chaperone holdase maintains polypeptides in soluble states for proteasome degradation.
The tissue-specific Rep8/UBXD6 tethers p97 to the endoplasmic reticulum membrane for degradation of misfolded proteins.
The unfolded protein response: integrating stress signals through the stress sensor IRE1α.
Defining human ERAD networks through an integrative mapping strategy.
Ubiquitin-specific protease 25 functions in Endoplasmic Reticulum-associated degradation.
STT3B-dependent posttranslational N-glycosylation as a surveillance system for secretory protein.
A shared endoplasmic reticulum-associated degradation pathway involving the EDEM1 protein for glycosylated and nonglycosylated proteins.
Endoplasmic reticulum stress and Parkinson's disease: the role of HRD1 in averting apoptosis in neurodegenerative disease.
A deubiquitinase negatively regulates retro-translocation of nonubiquitinated substrates.
Herp coordinates compartmentalization and recruitment of HRD1 and misfolded proteins for ERAD.
Identification of ERAD components essential for dislocation of the null Hong Kong variant of α-1-antitrypsin (NHK).
Association of the SEL1L protein transmembrane domain with HRD1 ubiquitin ligase regulates ERAD-L.
IRE1α is an endogenous substrate of endoplasmic-reticulum-associated degradation.
Conserved cytoplasmic domains promote Hrd1 ubiquitin ligase complex formation for ER-associated degradation (ERAD).
HSP70-Hrd1 axis precludes the oncorepressor potential of N-terminal misfolded Blimp-1s in lymphoma cells.
ER-localized Hrd1 ubiquitinates and inactivates Usp15 to promote TLR4-induced inflammation during bacterial infection.
Dual proteome-scale networks reveal cell-specific remodeling of the human interactome.
OpenCell: Endogenous tagging for the cartography of human cellular organization.
UFMylation of HRD1 regulates endoplasmic reticulum homeostasis.
Hypomorphic variants of SEL1L-HRD1 ER-associated degradation are associated with neurodevelopmental disorders.
Expression of SYVN1 (HRD1)
SYVN1 ubiquitinates Hh C-terminal fragments
C-terminal Hh fragments are recruited to SEL1:SYVN1 at the ER membrane
VCP-catalyzed ATP hydrolysis promotes the translocation of Hh-C into the cytosol
Hh processing variants are recruited to SEL1:SYVN at the ER membrane
Hh processing variants are translocated to the cytosol in a VCP-dependent manner
Hh processing variants are ubiquitinated
OS9:SEL1:ERAD E3 ligase:DERL2 ubiquitinates unfolded protein:(GlcNAc)2 (Man)9-5
ER Quality Control Compartment (ERQC)
SEL1L-HRD1 interaction is required to form a functional HRD1 ERAD complex.
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SEL1L-HRD1 physical interaction is a prerequisite for recruitment of E2 UBE2J1 and DERLIN proteins to HRD1, assembling a functional ERAD complex
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SEL1L hypomorphic variant S658P weakens SEL1L-HRD1 interaction (~5-fold) and causes partial embryonic lethality and cerebellar ataxia in mice
Hypomorphic variants of SEL1L-HRD1 ER-associated degradation are associated with neurodevelopmental disorders.
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Autosomal-recessive hypomorphic variants in HRD1/SYVN1 (including p.Pro398Leu) cause neurodevelopmental disorders with developmental delay, intellectual disability, and microcephaly in humans
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ERAD substrates IRE1alpha, OS9, and CD147 accumulate in knock-in cells with pathogenic HRD1 variants, confirming impaired ERAD function
Role of SYVN1 in the control of airway remodeling in asthma protection by promoting SIRT2 ubiquitination and degradation
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SYVN1 binds SIRT2 and promotes its ubiquitination and proteasome-dependent degradation in bronchial epithelial cells
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SYVN1 activity suppresses ER stress markers GRP78 and CHOP and epithelial-mesenchymal transition phenotypes through SIRT2 degradation
Synoviolin alleviates GSDMD-mediated periodontitis by suppressing its stability.
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Synoviolin/SYVN1 interacts with GSDMD and promotes its ubiquitination, modulating inflammasome-driven cytokine release
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Myeloid-specific Synoviolin conditional KO mice show greater bone loss in a periodontitis model
An evolutionarily conserved ubiquitin ligase drives infection and transmission of flaviviruses.
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HRD1 inhibitor LS-102 blocks HRD1 E3 activity in both mammalian and mosquito cells with EC50 values of 1.391 uM (A549) and 2.757 uM (C6/36)
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LS-102 treatment at 15 mg/kg in mice reduces dengue viremia and tissue viral burden ~10-fold with improved survival
A suite of genome-engineered hepatic cells provides novel insights into the spatiotemporal metabolism of apolipoprotein B and apolipoprotein B–containing lipoprotein secretion