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

Chemical stresses fail to mimic the unfolded protein response resulting from luminal load with unfolded polypeptides

(2018) Timothy J. Bergmann et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Myelin protein zero mutations and the unfolded protein response in Charcot Marie Tooth disease type 1B

(2018) Yunhong Bai et al.   Annals of Clinical and Translational Neurology

Impairment of protein degradation and proteasome function in hereditary neuropathies

(2017) Jordan J. S. VerPlank et al.   GLIA

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

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

Severity of Demyelinating and Axonal Neuropathy Mouse Models Is Modified by Genes Affecting Structure and Function of Peripheral Nodes

(2017) Kathryn H. Morelli et al.   Cell Reports

Endoplasmic Reticulum Protein Quality Control Failure in Myelin Disorders

(2017) Vera G. Volpi et al.   Frontiers in Molecular Neuroscience

Perk Ablation Ameliorates Myelination in S63del-Charcot–Marie–Tooth 1B Neuropathy

(2016) Nicolò Musner et al.   ASN Neuro

Endoplasmic reticulum stress and the unfolded protein response in disorders of myelinating glia

(2016) Benjamin L.L. Clayton et al.   BRAIN RESEARCH

Ultrasound pattern sum score, homogeneity score and regional nerve enlargement index for differentiation of demyelinating inflammatory and hereditary neuropathies

(2016) Alexander Grimm et al.   CLINICAL NEUROPHYSIOLOGY

The integrated stress response

(2016) Karolina Pakos‐Zebrucka et al.   EMBO REPORTS

Metabolic Reprogramming by Hexosamine Biosynthetic and Golgi N-Glycan Branching Pathways

(2016) Michael C. Ryczko et al.   Scientific Reports

Hereditary motor and sensory neuropathies: Understanding molecular pathogenesis could lead to future treatment strategies

(2015) Nivedita U. Jerath et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Myelin-associated glycoprotein gene mutation causes Pelizaeus-Merzbacher disease-like disorder

(2015) Alexander Lossos et al.   BRAIN

Hexosamine pathway and (ER) protein quality control

(2015) Martin S Denzel et al.   CURRENT OPINION IN CELL BIOLOGY

New insights on schwann cell development

(2015) Kelly R. Monk et al.   GLIA

ER Chaperone BiP/GRP78 Is Required for Myelinating Cell Survival and Provides Protection during Experimental Autoimmune Encephalomyelitis

(2015) Y. Hussien et al.   JOURNAL OF NEUROSCIENCE

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

(2015) Shengyi Sun et al.   NATURE CELL BIOLOGY

Proteostasis and aging

(2015) Susmita Kaushik et al.   NATURE MEDICINE

Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit

(2015) I. Das et al.   SCIENCE

Ubiquitin-specific protease 14 modulates degradation of cellular prion protein

(2015) Takujiro Homma et al.   Scientific Reports

Hexosamine Pathway Metabolites Enhance Protein Quality Control and Prolong Life

(2014) Martin S. Denzel et al.   CELL

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

(2014) Haibo Sha et al.   Cell Metabolism

Recent advances in Charcot–Marie–Tooth disease

(2014) Jonathan Baets et al.   CURRENT OPINION IN NEUROLOGY

Proteostasis and the Aging Proteome in Health and Disease

(2014) R. I. Morimoto et al.   JOURNALS OF GERONTOLOGY SERIES A-BIOLOGICAL SCIENCES AND MEDICAL SCIENCES

Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases

(2014) Claudio Hetz et al.   NATURE REVIEWS NEUROSCIENCE

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

Rer1 and calnexin regulate endoplasmic reticulum retention of a peripheral myelin protein 22 mutant that causes type 1A Charcot-Marie-Tooth disease

(2014) Taichi Hara et al.   Scientific Reports

Schwann cell phenotype is regulated by axon modality and central–peripheral location, and persists in vitro

(2013) T.M. Brushart et al.   EXPERIMENTAL NEUROLOGY

Resetting translational homeostasis restores myelination in Charcot-Marie-Tooth disease type 1B mice

(2013) Maurizio D’Antonio et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Stress-Independent Activation of XBP1s and/or ATF6 Reveals Three Functionally Diverse ER Proteostasis Environments

(2013) Matthew D. Shoulders et al.   Cell Reports

MpzR98C arrests Schwann cell development in a mouse model of early-onset Charcot–Marie–Tooth disease type 1B

(2012) Mario A. C. Saporta et al.   BRAIN

Phenotypic presentation of the Ser63Del MPZ mutation

(2012) Lindsey J. Miller et al.   JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM

Failure of Amino Acid Homeostasis Causes Cell Death following Proteasome Inhibition

(2012) Amila Suraweera et al.   MOLECULAR CELL

The Mammalian Endoplasmic Reticulum-Associated Degradation System

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

The endoplasmic reticulum stress response in aging and age-related diseases

(2012) Marishka K. Brown et al.   Frontiers in Physiology

Specialization of endoplasmic reticulum chaperones for the folding and function of myelin glycoproteins P0 and PMP22

(2011) Joanna Jung et al.   FASEB JOURNAL

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

P0 (Protein Zero) Mutation S34C Underlies Instability of Internodal Myelin in S63C Mice

(2010) Robin L. Avila et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Calnexin Deficiency Leads to Dysmyelination

(2010) Allison Kraus et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Myelination and support of axonal integrity by glia

(2010) Klaus-Armin Nave   NATURE

Enhancement of proteasome activity by a small-molecule inhibitor of USP14

(2010) Byung-Hoon Lee et al.   NATURE

Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane

(2010) T. Okiyoneda et al.   SCIENCE

Differences in endoplasmic-reticulum quality control determine the cellular response to disease-associated mutants of proteolipid protein

(2009) P. Roboti et al.   JOURNAL OF CELL SCIENCE

eIF2α kinases GCN2 and PERK modulate transcription and translation of distinct sets of mRNAs in mouse liver

(2009) An N. Dang Do et al.   PHYSIOLOGICAL GENOMICS

N-glycan structures: recognition and processing in the ER

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

Ablation of the UPR-Mediator CHOP Restores Motor Function and Reduces Demyelination in Charcot-Marie-Tooth 1B Mice

(2008) Maria Pennuto et al.   NEURON