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

A single point mutation in ricin A-chain increases toxin degradation and inhibits EDEM1-dependent ER retrotranslocation

(2011) Iwona Sokołowska et al.   BIOCHEMICAL JOURNAL

Generation of MHC class I ligands in the secretory and vesicular pathways

(2011) Margarita Del Val et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Protein folding and quality control in the endoplasmic reticulum: Recent lessons from yeast and mammalian cell systems

(2011) Jeffrey L Brodsky et al.   CURRENT OPINION IN CELL BIOLOGY

Protein folding stress in neurodegenerative diseases: a glimpse into the ER

(2011) Soledad Matus et al.   CURRENT OPINION IN CELL BIOLOGY

Regulating mitochondrial outer membrane proteins by ubiquitination and proteasomal degradation

(2011) Mariusz Karbowski et al.   CURRENT OPINION IN CELL BIOLOGY

Hyaline Fibromatosis Syndrome inducing mutations in the ectodomain of anthrax toxin receptor 2 can be rescued by proteasome inhibitors

(2011) Julie Deuquet et al.   EMBO Molecular Medicine

Mutant Atp13a2 proteins involved in parkinsonism are degraded by ER-associated degradation and sensitize cells to ER-stress induced cell death

(2011) Janet Ugolino et al.   HUMAN MOLECULAR GENETICS

Different p97/VCP complexes function in retrotranslocation step of mammalian Er-associated degradation (ERAD)

(2011) Petek Ballar et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

The Endoplasmic Reticulum (ER)-associated Degradation System Regulates Aggregation and Degradation of Mutant Neuroserpin

(2011) Zheng Ying et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A Unified View of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gating: Combining the Allosterism of a Ligand-gated Channel with the Enzymatic Activity of an ATP-binding Cassette (ABC) Transporter

(2011) Kevin L. Kirk et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Membrane-associated Ubiquitin Ligase Complex Containing gp78 Mediates Sterol-accelerated Degradation of 3-Hydroxy-3-methylglutaryl-coenzyme A Reductase

(2011) Youngah Jo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Probing Conformational Rescue Induced by a Chemical Corrector of F508del-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mutant

(2011) Wilson Yu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Role of the Endoplasmic Reticulum-associated Degradation (ERAD) Pathway in Degradation of Hepatitis C Virus Envelope Proteins and Production of Virus Particles

(2011) Mohsan Saeed et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Curcumin and genistein additively potentiate G551D-CFTR

(2011) Ying-Chun Yu et al.   Journal of Cystic Fibrosis

Inositol 1,4,5-trisphosphate receptors are essential for the development of the second heart field

(2011) Maki Nakazawa et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

Quantity Control of the ErbB3 Receptor Tyrosine Kinase at the Endoplasmic Reticulum

(2011) W. H. D. Fry et al.   MOLECULAR AND CELLULAR BIOLOGY

Bypass of glycan-dependent glycoprotein delivery to ERAD by up-regulated EDEM1

(2011) Efrat Ron et al.   MOLECULAR BIOLOGY OF THE CELL

A Ubiquitin Ligase-Associated Chaperone Holdase Maintains Polypeptides in Soluble States for Proteasome Degradation

(2011) Qiuyan Wang et al.   MOLECULAR CELL

Ligand-Driven Vectorial Folding of Ribosome-Bound Human CFTR NBD1

(2011) Amardeep Khushoo et al.   MOLECULAR CELL

Client-Loading Conformation of the Hsp90 Molecular Chaperone Revealed in the Cryo-EM Structure of the Human Hsp90:Hop Complex

(2011) Daniel R. Southworth et al.   MOLECULAR CELL

A Complex of Pdi1p and the Mannosidase Htm1p Initiates Clearance of Unfolded Glycoproteins from the Endoplasmic Reticulum

(2011) Robert Gauss et al.   MOLECULAR CELL

Structural Basis of an ERAD Pathway Mediated by the ER-Resident Protein Disulfide Reductase ERdj5

(2011) Masatoshi Hagiwara et al.   MOLECULAR CELL

A Therapeutic Chemical Chaperone Inhibits Cholera Intoxication and Unfolding/Translocation of the Cholera Toxin A1 Subunit

(2011) Michael Taylor et al.   PLoS One

HRD1 and UBE2J1 target misfolded MHC class I heavy chains for endoplasmic reticulum-associated degradation

(2011) Marian L. Burr et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Conformational dynamics of the molecular chaperone Hsp90

(2011) Kristin A. Krukenberg et al.   QUARTERLY REVIEWS OF BIOPHYSICS

Selective Inhibition of a Regulatory Subunit of Protein Phosphatase 1 Restores Proteostasis

(2011) P. Tsaytler et al.   SCIENCE

Molecular Mechanism of Co-translational Protein Targeting by the Signal Recognition Particle

(2011) Ishu Saraogi et al.   TRAFFIC

Alzheimer's Disease

(2011) D. J. Selkoe   Cold Spring Harbor Perspectives in Biology

TorsinA participates in endoplasmic reticulum-associated degradation

(2011) Flávia C. Nery et al.   Nature Communications

Ubiquitylation in ERAD: Reversing to Go Forward?

(2011) Yien Che Tsai et al.   PLOS BIOLOGY

Enzymatic Blockade of the Ubiquitin-Proteasome Pathway

(2011) Robert Ernst et al.   PLOS BIOLOGY

Pathogenic VCP/TER94 Alleles Are Dominant Actives and Contribute to Neurodegeneration by Altering Cellular ATP Level in a Drosophila IBMPFD Model

(2011) Ya-Chu Chang et al.   PLoS Genetics

Ca2+Homeostasis Modulation Enhances the Amenability of L444P Glucosylcerebrosidase to Proteostasis Regulation in Patient-Derived Fibroblasts

(2010) Fan Wang et al.   ACS Chemical Biology

Homozygosity for a Missense Mutation in SERPINH1, which Encodes the Collagen Chaperone Protein HSP47, Results in Severe Recessive Osteogenesis Imperfecta

(2010) Helena E. Christiansen et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Congenital disorders of glycosylation

(2010) Jaak Jaeken   Annals of the New York Academy of Sciences

VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD

(2010) Emilie Tresse et al.   Autophagy

Posttranslational negative regulation of glycosylated and non-glycosylated BCRP expression by Derlin-1

(2010) Takashi Sugiyama et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Protein dislocation from the ER

(2010) Katrin Bagola et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Clearance of RhodopsinP23H aggregates requires the ERAD effector VCP

(2010) Ana Griciuc et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Characterization of the ERAD process of the L444P mutant glucocerebrosidase variant

(2010) Inna Bendikov-Bar et al.   BLOOD CELLS MOLECULES AND DISEASES

Endoplasmic reticulum stress response in an INS-1 pancreatic β-cell line with inducible expression of a folding-deficient proinsulin

(2010) Taila Hartley et al.   BMC CELL BIOLOGY

Curcumin as a therapeutic agent: the evidence from in vitro, animal and human studies

(2010) Jenny Epstein et al.   BRITISH JOURNAL OF NUTRITION

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

(2010) Pedro Carvalho et al.   CELL

Ubiquitin: Same Molecule, Different Degradation Pathways

(2010) Michael J. Clague et al.   CELL

Endoplasmic Reticulum Stress and the Inflammatory Basis of Metabolic Disease

(2010) Gökhan S. Hotamisligil   CELL

Parkin is transcriptionally regulated by ATF4: evidence for an interconnection between mitochondrial stress and ER stress

(2010) L Bouman et al.   CELL DEATH AND DIFFERENTIATION

Coronaviruses Hijack the LC3-I-Positive EDEMosomes, ER-Derived Vesicles Exporting Short-Lived ERAD Regulators, for Replication

(2010) Fulvio Reggiori et al.   Cell Host & Microbe

A Novel ER J-protein DNAJB12 Accelerates ER-associated Degradation of Membrane Proteins Including CFTR

(2010) Yo-hei Yamamoto et al.   CELL STRUCTURE AND FUNCTION

The Role of Endoplasmic Reticulum Stress in the Progression of Atherosclerosis

(2010) Ira Tabas   CIRCULATION RESEARCH

Novel exon nucleotide substitution at the splice junction causes a neonatal Marfan syndrome

(2010) S-C Chao et al.   CLINICAL GENETICS

Chemical and/or biological therapeutic strategies to ameliorate protein misfolding diseases

(2010) Derrick Sek Tong Ong et al.   CURRENT OPINION IN CELL BIOLOGY

PERK (EIF2AK3) Regulates Proinsulin Trafficking and Quality Control in the Secretory Pathway

(2010) Sounak Gupta et al.   DIABETES

The endoplasmic reticulum stress response in the pancreatic β-cell

(2010) A. Volchuk et al.   DIABETES OBESITY & METABOLISM

Interaction between parkin and mutant glucocerebrosidase variants: a possible link between Parkinson disease and Gaucher disease

(2010) I. Ron et al.   HUMAN MOLECULAR GENETICS

Liver Cytochrome P450 3A Ubiquitinationin Vivoby gp78/Autocrine Motility Factor Receptor and C Terminus of Hsp70-interacting Protein (CHIP) E3 Ubiquitin Ligases

(2010) Sung-Mi Kim et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Molecular Characterization of the NPC1L1 Variants Identified from Cholesterol Low Absorbers

(2010) Li-Juan Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Herp Regulates Hrd1-mediated Ubiquitylation in a Ubiquitin-like Domain-dependent Manner

(2010) Melanie Kny et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hsp90 Is Required for Transfer of the Cholera Toxin A1 Subunit from the Endoplasmic Reticulum to the Cytosol

(2010) Michael Taylor et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Serine Residues in the Cytosolic Tail of the T-cell Antigen Receptor α-Chain Mediate Ubiquitination and Endoplasmic Reticulum-associated Degradation of the Unassembled Protein

(2010) Shuhei Ishikura et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Cystic Fibrosis-causing Mutation ΔF508 Affects Multiple Steps in Cystic Fibrosis Transmembrane Conductance Regulator Biogenesis

(2010) Patrick H. Thibodeau et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mannose Trimming Is Required for Delivery of a Glycoprotein from EDEM1 to XTP3-B and to Late Endoplasmic Reticulum-associated Degradation Steps

(2010) Bella Groisman et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A naturally occurring isoform inhibits parathyroid hormone receptor trafficking and signaling

(2010) Verónica Alonso et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Culling sick mitochondria from the herd: Figure 1.

(2010) Leo J. Pallanck   JOURNAL OF CELL BIOLOGY

Signal sequence insufficiency contributes to neurodegeneration caused by transmembrane prion protein

(2010) Neena S. Rane et al.   JOURNAL OF CELL BIOLOGY

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

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

A novel GJB1 frameshift mutation produces a transient CNS symptom of X-linked Charcot–Marie–Tooth disease

(2010) Hideya Sakaguchi et al.   JOURNAL OF NEUROLOGY

Interactions of heat shock protein 47 with collagen and the stress response: An unconventional chaperone model?

(2010) J. Geraldine Sandana Mala et al.   LIFE SCIENCES

The ubiquitin ligase Hrd1 promotes degradation of the Z variant alpha 1-antitrypsin and increases its solubility

(2010) Haiping Wang et al.   MOLECULAR AND CELLULAR BIOCHEMISTRY

Folding-competent and Folding-defective Forms of Ricin A Chain Have Different Fates after Retrotranslocation from the Endoplasmic Reticulum

(2010) Shuyu Li et al.   MOLECULAR BIOLOGY OF THE CELL

Biological and Structural Basis for Aha1 Regulation of Hsp90 ATPase Activity in Maintaining Proteostasis in the Human Disease Cystic Fibrosis

(2010) Atanas V. Koulov et al.   MOLECULAR BIOLOGY OF THE CELL

Role of Hsp90 in Biogenesis of the β-Cell ATP-sensitive Potassium Channel Complex

(2010) Fei-Fei Yan et al.   MOLECULAR BIOLOGY OF THE CELL

BiP Availability Distinguishes States of Homeostasis and Stress in the Endoplasmic Reticulum of Living Cells

(2010) Chun Wei Lai et al.   MOLECULAR BIOLOGY OF THE CELL

The Ero1α-PDI Redox Cycle Regulates Retro-Translocation of Cholera Toxin

(2010) Paul Moore et al.   MOLECULAR BIOLOGY OF THE CELL

The endoplasmic reticulum–associated Hsp40 DNAJB12 and Hsc70 cooperate to facilitate RMA1 E3–dependent degradation of nascent CFTRΔF508

(2010) Diane E. Grove et al.   MOLECULAR BIOLOGY OF THE CELL

The AAA-ATPase p97 is essential for outer mitochondrial membrane protein turnover

(2010) Shan Xu et al.   MOLECULAR BIOLOGY OF THE CELL

Ubiquitylation of an ERAD Substrate Occurs on Multiple Types of Amino Acids

(2010) Yuichiro Shimizu et al.   MOLECULAR CELL

A Stress-Responsive System for Mitochondrial Protein Degradation

(2010) Jin-Mi Heo et al.   MOLECULAR CELL

An ITPR1 gene deletion causes spinocerebellar ataxia 15/16: A genetic, clinical and radiological description

(2010) Marianne J.U. Novak et al.   MOVEMENT DISORDERS

Endoplasmic reticulum Ca2+ increases enhance mutant glucocerebrosidase proteostasis

(2010) Derrick Sek Tong Ong et al.   Nature Chemical Biology

Mechanisms of mitophagy

(2010) Richard J. Youle et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mammal-restricted elements predispose human RET to folding impairment by HSCR mutations

(2010) Svend Kjær et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Huntingtin Interacts with the Cue Domain of gp78 and Inhibits gp78 Binding to Ubiquitin and p97/VCP

(2010) Hui Yang et al.   PLoS One

Clinical and molecular genetics of neonatal diabetes due to mutations in the insulin gene

(2010) Julie Støy et al.   REVIEWS IN ENDOCRINE & METABOLIC DISORDERS

An Autophagy-Enhancing Drug Promotes Degradation of Mutant  1-Antitrypsin Z and Reduces Hepatic Fibrosis

(2010) T. Hidvegi et al.   SCIENCE

Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane

(2010) T. Okiyoneda et al.   SCIENCE

ER quality control in the biogenesis of MHC class I molecules

(2010) Daniel C. Chapman et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Stress hypERactivation in the β-cell

(2010) Sonya G. Fonseca et al.   Islets

BiP Binding to the ER-Stress Sensor Ire1 Tunes the Homeostatic Behavior of the Unfolded Protein Response

(2010) David Pincus et al.   PLOS BIOLOGY

Inactivation of VCP/ter94 Suppresses Retinal Pathology Caused by Misfolded Rhodopsin in Drosophila

(2010) Ana Griciuc et al.   PLoS Genetics

Multilayered Mechanism of CD4 Downregulation by HIV-1 Vpu Involving Distinct ER Retention and ERAD Targeting Steps

(2010) Javier G. Magadán et al.   PLoS Pathogens

The ER and ageing II: Calcium homeostasis

(2009) Monika Puzianowska-Kuznicka et al.   AGEING RESEARCH REVIEWS

Biological and Chemical Approaches to Diseases of Proteostasis Deficiency

(2009) Evan T. Powers et al.   Annual Review of Biochemistry

Conformational Pathology of the Serpins: Themes, Variations, and Therapeutic Strategies

(2009) Bibek Gooptu et al.   Annual Review of Biochemistry

Recognition and Processing of Ubiquitin-Protein Conjugates by the Proteasome

(2009) Daniel Finley   Annual Review of Biochemistry

Protein Disulfide Isomerase: A Critical Evaluation of Its Function in Disulfide Bond Formation

(2009) Feras Hatahet et al.   ANTIOXIDANTS & REDOX SIGNALING

Mutant proinsulin proteins associated with neonatal diabetes are retained in the endoplasmic reticulum and not efficiently secreted

(2009) Soo-Young Park et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

PtdIns(3,4,5)P3and inositol depletion as a cellular target of mood stabilizers

(2009) Regina Teo et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Defining the Human Deubiquitinating Enzyme Interaction Landscape

(2009) Mathew E. Sowa et al.   CELL

Quantitative Proteomics Reveals the Function of Unconventional Ubiquitin Chains in Proteasomal Degradation

(2009) Ping Xu et al.   CELL

When worlds collide: IP3 receptors and the ERAD pathway

(2009) Richard J.H. Wojcikiewicz et al.   CELL CALCIUM

α1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies

(2009) Ugo I. Ekeowa et al.   CLINICAL SCIENCE

Diagnosis and Classification of Diabetes Mellitus

(2009)   DIABETES CARE

Protein disulphide isomerase is required for signal peptide peptidase-mediated protein degradation

(2009) Seong-Ok Lee et al.   EMBO JOURNAL

Disruption of N-linked glycosylation enhances ubiquitin-mediated proteasomal degradation of the human ATP-binding cassette transporter ABCG2

(2009) Hiroshi Nakagawa et al.   FEBS Journal

Folding defects in P-type ATP 8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate

(2009) Lieke M. van der Velden et al.   HEPATOLOGY

Differential effects of progressive familial intrahepatic cholestasis type 1 and benign recurrent intrahepatic cholestasis type 1 mutations on canalicular localization of ATP8B1

(2009) Dineke E. Folmer et al.   HEPATOLOGY

PRKCSH/80K-H, the protein mutated in polycystic liver disease, protects polycystin-2/TRPP2 against HERP-mediated degradation

(2009) Hongyu Gao et al.   HUMAN MOLECULAR GENETICS

Gp78, an ER associated E3, promotes SOD1 and ataxin-3 degradation

(2009) Zheng Ying et al.   HUMAN MOLECULAR GENETICS

Genetic Modifiers of Liver Disease in Cystic Fibrosis

(2009) Jaclyn R. Bartlett   JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION

Roles of Protein-disulfide Isomerase-mediated Disulfide Bond Formation of Yeast Mnl1p in Endoplasmic Reticulum-associated Degradation

(2009) Machiko Sakoh-Nakatogawa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Meckel-Gruber Syndrome Protein MKS3 Is Required for Endoplasmic Reticulum-associated Degradation of Surfactant Protein C

(2009) Mei Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hsp40 Chaperones Promote Degradation of the hERG Potassium Channel

(2009) Valerie E. Walker et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Generating an Unfoldase from Thioredoxin-like Domains

(2009) Michele L. Forster et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

In VitroAnalysis of Hrd1p-mediated Retrotranslocation of Its Multispanning Membrane Substrate 3-Hydroxy-3-methylglutaryl (HMG)-CoA Reductase

(2009) Renee M. Garza et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Endoplasmic Reticulum-associated Degradation (ERAD) and Autophagy Cooperate to Degrade Polymerogenic Mutant Serpins

(2009) Heike Kroeger et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

N-glycans are direct determinants of CFTR folding and stability in secretory and endocytic membrane traffic

(2009) Rina Glozman et al.   JOURNAL OF CELL BIOLOGY

The TRC8 E3 ligase ubiquitinates MHC class I molecules before dislocation from the ER

(2009) Helen R. Stagg et al.   JOURNAL OF CELL BIOLOGY

Ube2j2 ubiquitinates hydroxylated amino acids on ER-associated degradation substrates

(2009) Xiaoli Wang et al.   JOURNAL OF CELL BIOLOGY

Htm1 protein generates the N-glycan signal for glycoprotein degradation in the endoplasmic reticulum

(2009) Simone Clerc et al.   JOURNAL OF CELL BIOLOGY

A dual role for EDEM1 in the processing of rod opsin

(2009) Maria Kosmaoglou et al.   JOURNAL OF CELL SCIENCE

The pharmacological chaperone 1-deoxygalactonojirimycin increases α-galactosidase A levels in Fabry patient cell lines

(2009) E. R. Benjamin et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Aging-dependent changes of microglial cells and their relevance for neurodegenerative disorders

(2009) Rommy von Bernhardi et al.   JOURNAL OF NEUROCHEMISTRY

Two Molecular Pathways (NMD and ERAD) Contribute to a Genetic Epilepsy Associated with the GABAA Receptor GABRA1 PTC Mutation, 975delC, S326fs328X

(2009) J.-Q. Kang et al.   JOURNAL OF NEUROSCIENCE

The GABRG2 Mutation, Q351X, Associated with Generalized Epilepsy with Febrile Seizures Plus, Has Both Loss of Function and Dominant-Negative Suppression

(2009) J.-Q. Kang et al.   JOURNAL OF NEUROSCIENCE

Mechanisms for Rescue of Correctable Folding Defects in CFTRΔF508

(2009) Diane E. Grove et al.   MOLECULAR BIOLOGY OF THE CELL

Cooperative Assembly and Misfolding of CFTR Domains In Vivo

(2009) Kai Du et al.   MOLECULAR BIOLOGY OF THE CELL

Dislocation of HMG-CoA Reductase and Insig-1, Two Polytopic Endoplasmic Reticulum Proteins, En Route to Proteasomal Degradation

(2009) Gil S. Leichner et al.   MOLECULAR BIOLOGY OF THE CELL

Autophagic Elimination of Misfolded Procollagen Aggregates in the Endoplasmic Reticulum as a Means of Cell Protection

(2009) Yoshihito Ishida et al.   MOLECULAR BIOLOGY OF THE CELL

The E3 Ubiquitin Ligases Hrd1 and gp78 Bind to and Promote Cholera Toxin Retro-Translocation

(2009) Kaleena M. Bernardi et al.   MOLECULAR BIOLOGY OF THE CELL

EDEM1 Recognition and Delivery of Misfolded Proteins to the SEL1L-Containing ERAD Complex

(2009) James H. Cormier et al.   MOLECULAR CELL

Misfolded Membrane Proteins Are Specifically Recognized by the Transmembrane Domain of the Hrd1p Ubiquitin Ligase

(2009) Brian K. Sato et al.   MOLECULAR CELL

Human genetics illuminates the paths to metabolic disease

(2009) Stephen O’Rahilly   NATURE

The ubiquitylation machinery of the endoplasmic reticulum

(2009) Christian Hirsch et al.   NATURE

Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis

(2009) Darren M Hutt et al.   Nature Chemical Biology

Calcium Pumps in Health and Disease

(2009) Marisa Brini et al.   PHYSIOLOGICAL REVIEWS

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

(2009) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ERAD substrates: Which way out?

(2009) Daniel N. Hebert et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Intracellular Assembly and Trafficking of MHC Class I Molecules

(2009) Julie G. Donaldson et al.   TRAFFIC

Prion protein biosynthesis and its emerging role in neurodegeneration

(2009) Oishee Chakrabarti et al.   TRENDS IN BIOCHEMICAL SCIENCES

N-glycan structures: recognition and processing in the ER

(2009) Markus Aebi et al.   TRENDS IN BIOCHEMICAL SCIENCES

Inhibition of Proteasome Activity Promotes the Correct Localization of Disease-Causing α-Sarcoglycan Mutants in HEK-293 Cells Constitutively Expressing β-, γ-, and δ-Sarcoglycan

(2008) Stefano Gastaldello et al.   AMERICAN JOURNAL OF PATHOLOGY

CFTR Function and Prospects for Therapy

(2008) John R. Riordan   Annual Review of Biochemistry

The Protein Folding Problem

(2008) Ken A. Dill et al.   Annual Review of Biophysics

Histones: Annotating Chromatin

(2008) Eric I. Campos et al.   Annual Review of Genetics

Endoplasmic Reticulum Stress in Disease Pathogenesis

(2008) Jonathan H. Lin et al.   Annual Review of Pathology-Mechanisms of Disease

Ubiquitin-mediated proteasomal degradation of non-synonymous SNP variants of human ABC transporter ABCG2

(2008) Hiroshi Nakagawa et al.   BIOCHEMICAL JOURNAL

Rescue of mutant α-galactosidase A in the endoplasmic reticulum by 1-deoxygalactonojirimycin leads to trafficking to lysosomes

(2008) Ryoji Hamanaka et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Inositol trisphosphate and calcium signalling mechanisms

(2008) Michael J. Berridge   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Chemical and Biological Approaches Synergize to Ameliorate Protein-Folding Diseases

(2008) Ting-Wei Mu et al.   CELL

Dissecting the ER-Associated Degradation of a Misfolded Polytopic Membrane Protein

(2008) Kunio Nakatsukasa et al.   CELL

UBX domain proteins: major regulators of the AAA ATPase Cdc48/p97

(2008) C. Schuberth et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Mechanisms of neurodegeneration in Huntington’s disease

(2008) Joana M. Gil et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Protein folding includes oligomerization - examples from the endoplasmic reticulum and cytosol

(2008) Chantal Christis et al.   FEBS Journal

Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity

(2008) M. L. Duennwald et al.   GENES & DEVELOPMENT

Degradation of the bile salt export pump at endoplasmic reticulum in progressive familial intrahepatic cholestasis type II

(2008) Lin Wang et al.   HEPATOLOGY

Polycystin-2 is regulated by endoplasmic reticulum-associated degradation

(2008) G. Liang et al.   HUMAN MOLECULAR GENETICS

Dystonia-associated mutations cause premature degradation of torsinA protein and cell-type-specific mislocalization to the nuclear envelope

(2008) L. M. Giles et al.   HUMAN MOLECULAR GENETICS

Discovery of Cellular Regulation by Protein Degradation

(2008) Alexander Varshavsky   JOURNAL OF BIOLOGICAL CHEMISTRY

Processing Mutations Disrupt Interactions between the Nucleotide Binding and Transmembrane Domains of P-glycoprotein and the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

(2008) Tip W. Loo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Niemann-Pick Type C1 I1061T Mutant Encodes a Functional Protein That Is Selected for Endoplasmic Reticulum-associated Degradation Due to Protein Misfolding

(2008) Mark E. Gelsthorpe et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mechanisms Involved in the Reduction of GABAAReceptor α1-Subunit Expression Caused by the Epilepsy Mutation A322D in the Trafficking-competent Receptor

(2008) Clarrisa A. Bradley et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Human XTP3-B Forms an Endoplasmic Reticulum Quality Control Scaffold with the HRD1-SEL1L Ubiquitin Ligase Complex and BiP

(2008) Nobuko Hosokawa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Hsp90 Chaperone Machinery

(2008) Sebastian Karl Wandinger et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Sec61p Is Required for ERAD-L

(2008) Martin Willer et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Role of Sec61p in the ER-associated degradation of short-lived transmembrane proteins

(2008) Daniel C. Scott et al.   JOURNAL OF CELL BIOLOGY

Immunohistochemical localization of a ubiquitin ligase HRD1 in murine brain

(2008) Tomohiro Omura et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Gp78 Cooperates with RMA1 in Endoplasmic Reticulum-associated Degradation of CFTRΔF508

(2008) Daisuke Morito et al.   MOLECULAR BIOLOGY OF THE CELL

Assembly and Misassembly of Cystic Fibrosis Transmembrane Conductance Regulator: Folding Defects Caused by Deletion of F508 Occur Before and After the Calnexin-dependent Association of Membrane Spanning Domain (MSD) 1 and MSD2

(2008) Meredith F. N. Rosser et al.   MOLECULAR BIOLOGY OF THE CELL

ERdj4 and ERdj5 Are Required for Endoplasmic Reticulum-associated Protein Degradation of Misfolded Surfactant Protein C

(2008) Mei Dong et al.   MOLECULAR BIOLOGY OF THE CELL

Ubiquitin–Proteasome-dependent Degradation of a Mitofusin, a Critical Regulator of Mitochondrial Fusion

(2008) Mickael M.J. Cohen et al.   MOLECULAR BIOLOGY OF THE CELL

Defining the Glycan Destruction Signal for Endoplasmic Reticulum-Associated Degradation

(2008) Erin M. Quan et al.   MOLECULAR CELL

Ubiquitin docking at the proteasome through a novel pleckstrin-homology domain interaction

(2008) Patrick Schreiner et al.   NATURE

Proteasome subunit Rpn13 is a novel ubiquitin receptor

(2008) Koraljka Husnjak et al.   NATURE

From endoplasmic-reticulum stress to the inflammatory response

(2008) Kezhong Zhang et al.   NATURE

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

The many roles of histone deacetylases in development and physiology: implications for disease and therapy

(2008) Michael Haberland et al.   NATURE REVIEWS GENETICS

Major SNP (Q141K) Variant of Human ABC Transporter ABCG2 Undergoes Lysosomal and Proteasomal Degradations

(2008) Tomoka Furukawa et al.   PHARMACEUTICAL RESEARCH

Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function

(2008) A. W. R. Serohijos et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The human PDI family: Versatility packed into a single fold

(2007) Christian Appenzeller-Herzog et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Mechanisms of Aβ mediated neurodegeneration in Alzheimer's disease

(2007) Peter J. Crouch et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Derlin-1 Facilitates the Retro-Translocation of Cholera Toxin

(2007) Kaleena M. Bernardi et al.   MOLECULAR BIOLOGY OF THE CELL

Intracellular cholesterol modifies the ERAD of glucocerebrosidase in Gaucher disease patients☆

(2007) Idit Ron et al.   MOLECULAR GENETICS AND METABOLISM