ER-associated degradation in health and disease – from substrate to organism

Endoplasmic Reticulum Associated Degradation (ERAD) Has a Critical Role in Supporting Glucose-Stimulated Insulin Secretion in Pancreatic β-Cells

(2019) Yabing Hu et al.   DIABETES

IRE1α Activation in Bone Marrow-Derived Dendritic Cells Modulates Innate Recognition of Melanoma Cells and Favors CD8+ T Cell Priming

(2019) Bernardita Medel et al.   Frontiers in Immunology

Schwann cells ER-associated degradation contributes to myelin maintenance in adult nerves and limits demyelination in CMT1B mice

(2019) Vera G. Volpi et al.   PLoS Genetics

The orphan nuclear receptor SHP regulates ER stress response by inhibiting XBP1s degradation

(2019) Shengyi Sun et al.   GENES & DEVELOPMENT

AIDA Selectively Mediates Downregulation of Fat Synthesis Enzymes by ERAD to Retard Intestinal Fat Absorption and Prevent Obesity

(2018) Hui Luo et al.   Cell Metabolism

The endoplasmic reticulum-–resident E3 ubiquitin ligase Hrd1 controls a critical checkpoint in B- cell development in mice

(2018) Yi Yang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

IRE1–XBP1 pathway regulates oxidative proinsulin folding in pancreatic β cells

(2018) Yuichi Tsuchiya et al.   JOURNAL OF CELL BIOLOGY

Hypothalamic ER–associated degradation regulates POMC maturation, feeding, and age-associated obesity

(2018) Geun Hyang Kim et al.   JOURNAL OF CLINICAL INVESTIGATION

Redundant and Antagonistic Roles of XTP3B and OS9 in Decoding Glycan and Non-glycan Degrons in ER-Associated Degradation

(2018) Annemieke T. van der Goot et al.   MOLECULAR CELL

IRE1α prevents hepatic steatosis by processing and promoting the degradation of select microRNAs

(2018) Jie-Mei Wang et al.   Science Signaling

Quality Control in the Endoplasmic Reticulum: Crosstalk between ERAD and UPR pathways

(2018) Jiwon Hwang et al.   TRENDS IN BIOCHEMICAL SCIENCES

IRE1α prevents hepatic steatosis by processing and promoting the degradation of select microRNAs

(2018) Jie-Mei Wang et al.   Science Signaling

Adipocyte Xbp1s overexpression drives uridine production and reduces obesity

(2018) Yingfeng Deng et al.   Molecular Metabolism

Underlying mechanisms for sterol-induced ubiquitination and ER-associated degradation of HMG CoA reductase

(2018) Brittany M. Johnson et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

ER-associated ubiquitin ligase HRD1 programs liver metabolism by targeting multiple metabolic enzymes

(2018) Juncheng Wei et al.   Nature Communications

HRD1‐ERAD controls production of the hepatokine FGF21 through CREBH polyubiquitination

(2018) Juncheng Wei et al.   EMBO JOURNAL

Hepatic Sel1L‐Hrd1 ER‐associated degradation (ERAD) manages FGF21 levels and systemic metabolism via CREBH

(2018) Asmita Bhattacharya et al.   EMBO JOURNAL

ER-associated degradation is required for vasopressin prohormone processing and systemic water homeostasis

(2017) Guojun Shi et al.   JOURNAL OF CLINICAL INVESTIGATION

The p97 Inhibitor CB-5083 Is a Unique Disrupter of Protein Homeostasis in Models of Multiple Myeloma

(2017) Ronan Le Moigne et al.   MOLECULAR CANCER THERAPEUTICS

New Insights into the Physiological Role of Endoplasmic Reticulum-Associated Degradation

(2017) Ling Qi et al.   TRENDS IN CELL BIOLOGY

Autoubiquitination of the Hrd1 Ligase Triggers Protein Retrotranslocation in ERAD

(2016) Ryan D. Baldridge et al.   CELL

Ire1α in Pomc Neurons Is Required for Thermogenesis and Glycemia

(2016) Ting Yao et al.   DIABETES

Epithelial Sel1L is required for the maintenance of intestinal homeostasis

(2016) Shengyi Sun et al.   MOLECULAR BIOLOGY OF THE CELL

Direct and essential function for Hrd3 in ER-associated degradation

(2016) Nidhi Vashistha et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Endoplasmic reticulum-resident E3 ubiquitin ligase Hrd1 controls B-cell immunity through degradation of the death receptor CD95/Fas

(2016) Sinyi Kong et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The ER membrane-anchored ubiquitin ligase Hrd1 is a positive regulator of T-cell immunity

(2016) Yuanming Xu et al.   Nature Communications

The Sel1L-Hrd1 Endoplasmic Reticulum-Associated Degradation Complex Manages a Key Checkpoint in B Cell Development

(2016) Yewei Ji et al.   Cell Reports

Knockout of inositol-requiring enzyme 1α in pro-opiomelanocortin neurons decreases fat mass via increasing energy expenditure

(2016) Yuzhong Xiao et al.   Open Biology

Targeting the AAA ATPase p97 as an Approach to Treat Cancer through Disruption of Protein Homeostasis

(2015) Daniel J. Anderson et al.   CANCER CELL

The E3 ligase synoviolin controls body weight and mitochondrial biogenesis through negative regulation of PGC-1 

(2015) H. Fujita et al.   EMBO JOURNAL

gp78 functions downstream of Hrd1 to promote degradation of misfolded proteins of the endoplasmic reticulum

(2015) Ting Zhang et al.   MOLECULAR BIOLOGY OF THE CELL

IRE1α is an endogenous substrate of endoplasmic-reticulum-associated degradation

(2015) Shengyi Sun et al.   NATURE CELL BIOLOGY

NRF2 regulates serine biosynthesis in non–small cell lung cancer

(2015) Gina M DeNicola et al.   NATURE GENETICS

FGF21 Acts Centrally to Induce Sympathetic Nerve Activity, Energy Expenditure, and Weight Loss

(2014) Bryn M. Owen et al.   Cell Metabolism

The ER-Associated Degradation Adaptor Protein Sel1L Regulates LPL Secretion and Lipid Metabolism

(2014) Haibo Sha et al.   Cell Metabolism

The IRE1α-XBP1 pathway regulates metabolic stress-induced compensatory proliferation of pancreatic β-cells

(2014) Tongfu Xu et al.   CELL RESEARCH

Hrd1 suppresses Nrf2-mediated cellular protection during liver cirrhosis

(2014) T. Wu et al.   GENES & DEVELOPMENT

Fibroblast Growth Factor 21 Is Regulated by the IRE1α-XBP1 Branch of the Unfolded Protein Response and Counteracts Endoplasmic Reticulum Stress-induced Hepatic Steatosis

(2014) Shan Jiang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hrd1-mediated BLIMP-1 ubiquitination promotes dendritic cell MHCII expression for CD4 T cell priming during inflammation

(2014) Heeyoung Yang et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Cleaning up in the endoplasmic reticulum: ubiquitin in charge

(2014) John C Christianson et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Sel1L is indispensable for mammalian endoplasmic reticulum-associated degradation, endoplasmic reticulum homeostasis, and survival

(2014) Shengyi Sun et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Hepatic IRE1α regulates fasting-induced metabolic adaptive programs through the XBP1s–PPARα axis signalling

(2014) Mengle Shao et al.   Nature Communications

Adipocyte Spliced Form of X-Box-Binding Protein 1 Promotes Adiponectin Multimerization and Systemic Glucose Homeostasis

(2013) H. Sha et al.   DIABETES

Regulated IRE1-dependent decay participates in curtailing immunoglobulin secretion from plasma cells

(2013) Sandrine Benhamron et al.   EUROPEAN JOURNAL OF IMMUNOLOGY

Phenformin Activates the Unfolded Protein Response in an AMP-activated Protein Kinase (AMPK)-dependent Manner

(2013) Liu Yang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

ER stress transcription factor Xbp1 suppresses intestinal tumorigenesis and directs intestinal stem cells

(2013) Lukas Niederreiter et al.   JOURNAL OF EXPERIMENTAL MEDICINE

FGF21 contributes to neuroendocrine control of female reproduction

(2013) Bryn M Owen et al.   NATURE MEDICINE

Specificity and Regulation of the Endoplasmic Reticulum-Associated Degradation Machinery

(2013) Jessica Merulla et al.   TRAFFIC

Sterol homeostasis requires regulated degradation of squalene monooxygenase by the ubiquitin ligase Doa10/Teb4

(2013) Ombretta Foresti et al.   eLife

Ablation of gp78 in Liver Improves Hyperlipidemia and Insulin Resistance by Inhibiting SREBP to Decrease Lipid Biosynthesis

(2012) Tong-Fei Liu et al.   Cell Metabolism

Nonmuscle Myosin IIB Links Cytoskeleton to IRE1α Signaling during ER Stress

(2012) Yin He et al.   DEVELOPMENTAL CELL

FGF21 regulates PGC-1  and browning of white adipose tissues in adaptive thermogenesis

(2012) f. M. Fisher et al.   GENES & DEVELOPMENT

RING-finger type E3 ubiquitin ligase inhibitors as novel candidates for the treatment of rheumatoid arthritis

(2012) NAOKO YAGISHITA et al.   INTERNATIONAL JOURNAL OF MOLECULAR MEDICINE

Unassembled CD147 is an endogenous endoplasmic reticulum–associated degradation substrate

(2012) Ryan E. Tyler et al.   MOLECULAR BIOLOGY OF THE CELL

Differential regulation of HMG-CoA reductase and Insig-1 by enzymes of the ubiquitin-proteasome system

(2012) Yien Che Tsai et al.   MOLECULAR BIOLOGY OF THE CELL

Emerging functions of the VCP/p97 AAA-ATPase in the ubiquitin system

(2012) Hemmo Meyer et al.   NATURE CELL BIOLOGY

The Delicate Balance Between Secreted Protein Folding and Endoplasmic Reticulum-Associated Degradation in Human Physiology

(2012) Christopher J. Guerriero et al.   PHYSIOLOGICAL REVIEWS

Derlin-1 Deficiency Is Embryonic Lethal, Derlin-3 Deficiency Appears Normal, and Herp Deficiency Is Intolerant to Glucose Load and Ischemia in Mice

(2012) Yuka Eura et al.   PLoS One

The Mammalian Endoplasmic Reticulum-Associated Degradation System

(2012) J. A. Olzmann et al.   Cold Spring Harbor Perspectives in Biology

Protein Folding and Modification in the Mammalian Endoplasmic Reticulum

(2011) Ineke Braakman et al.   Annual Review of Biochemistry

The unfolded protein response transducer IRE1α prevents ER stress-induced hepatic steatosis

(2011) Kezhong Zhang et al.   EMBO JOURNAL

Extensive Pancreas Regeneration Following Acinar-Specific Disruption of Xbp1 in Mice

(2011) David A. Hess et al.   GASTROENTEROLOGY

Derlin-2-Deficient Mice Reveal an Essential Role for Protein Dislocation in Chondrocytes

(2011) S. K. Dougan et al.   MOLECULAR AND CELLULAR BIOLOGY

Defining human ERAD networks through an integrative mapping strategy

(2011) John C. Christianson et al.   NATURE CELL BIOLOGY

Dual and opposing roles of the unfolded protein response regulated by IRE1  and XBP1 in proinsulin processing and insulin secretion

(2011) A.-H. Lee et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Road to Ruin: Targeting Proteins for Degradation in the Endoplasmic Reticulum

(2011) M. H. Smith et al.   SCIENCE

The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation

(2011) P. Walter et al.   SCIENCE

Protein Folding and Quality Control in the ER

(2011) K. Araki et al.   Cold Spring Harbor Perspectives in Biology

Retrotranslocation of a Misfolded Luminal ER Protein by the Ubiquitin-Ligase Hrd1p

(2010) Pedro Carvalho et al.   CELL

Hepatic FGF21 Expression Is Induced at Birth via PPARα in Response to Milk Intake and Contributes to Thermogenic Activation of Neonatal Brown Fat

(2010) Elayne Hondares et al.   Cell Metabolism

Quality and quantity control at the endoplasmic reticulum

(2010) Ramanujan S Hegde et al.   CURRENT OPINION IN CELL BIOLOGY

Deficiency of Suppressor Enhancer Lin12 1 Like (SEL1L) in Mice Leads to Systemic Endoplasmic Reticulum Stress and Embryonic Lethality

(2010) Adam B. Francisco et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Stringent requirement for HRD1, SEL1L, and OS-9/XTP3-B for disposal of ERAD-LSsubstrates

(2010) Riccardo Bernasconi et al.   JOURNAL OF CELL BIOLOGY

The Cavβ subunit prevents RFP2-mediated ubiquitination and proteasomal degradation of L-type channels

(2010) Christophe Altier et al.   NATURE NEUROSCIENCE

A Phos-Tag-Based Approach Reveals the Extent of Physiological Endoplasmic Reticulum Stress

(2010) Liu Yang et al.   PLoS One

The IRE1α-XBP1 Pathway of the Unfolded Protein Response Is Required for Adipogenesis

(2009) Haibo Sha et al.   Cell Metabolism

Dominant pro-vasopressin mutants that cause diabetes insipidus form disulfide-linked fibrillar aggregates in the endoplasmic reticulum

(2009) J. Birk et al.   JOURNAL OF CELL SCIENCE

De Novo Ceramide Synthesis Is Required for N-Linked Glycosylation in Plasma Cells

(2009) M. Goldfinger et al.   JOURNAL OF IMMUNOLOGY

The Otubain YOD1 Is a Deubiquitinating Enzyme that Associates with p97 to Facilitate Protein Dislocation from the ER

(2009) Robert Ernst et al.   MOLECULAR CELL

XBP1 Links ER Stress to Intestinal Inflammation and Confers Genetic Risk for Human Inflammatory Bowel Disease

(2008) Arthur Kaser et al.   CELL

Inhibition of Growth Hormone Signaling by the Fasting-Induced Hormone FGF21

(2008) Takeshi Inagaki et al.   Cell Metabolism

Mutations in the Amino-Terminal Region of Proopiomelanocortin (POMC) in Patients with Early-Onset Obesity Impair POMC Sorting to the Regulated Secretory Pathway

(2008) John W. M. Creemers et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

OS-9 and GRP94 deliver mutant α1-antitrypsin to the Hrd1–SEL1L ubiquitin ligase complex for ERAD

(2008) John C. Christianson et al.   NATURE CELL BIOLOGY

SEL1L nucleates a protein complex required for dislocation of misfolded glycoproteins

(2008) B. Mueller et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA