Impairment of protein degradation and proteasome function in hereditary neuropathies

Ablation of Perk in Schwann Cells Improves Myelination in the S63del Charcot-Marie-Tooth 1B Mouse

(2016) M. Sidoli et al.   JOURNAL OF NEUROSCIENCE

Control of proteasomal proteolysis by mTOR

(2016) Jinghui Zhao et al.   NATURE

USP14 deubiquitinates proteasome-bound substrates that are ubiquitinated at multiple sites

(2016) Byung-Hoon Lee et al.   NATURE

Genotype–phenotype characteristics and baseline natural history of heritable neuropathies caused by mutations in theMPZgene

(2015) Oranee Sanmaneechai et al.   BRAIN

Tau-driven 26S proteasome impairment and cognitive dysfunction can be prevented early in disease by activating cAMP-PKA signaling

(2015) Natura Myeku et al.   NATURE MEDICINE

cAMP-induced phosphorylation of 26S proteasomes on Rpn6/PSMD11 enhances their activity and the degradation of misfolded proteins

(2015) Sudarsanareddy Lokireddy et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structural characterization of the interaction of Ubp6 with the 26S proteasome

(2015) Antje Aufderheide et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

mTOR inhibition activates overall protein degradation by the ubiquitin proteasome system as well as by autophagy

(2015) Jinghui Zhao et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Proteasome-Mediated Processing of Nrf1 Is Essential for Coordinate Induction of All Proteasome Subunits and p97

(2014) Zhe Sha et al.   CURRENT BIOLOGY

Autoubiquitination of the 26S Proteasome on Rpn13 Regulates Breakdown of Ubiquitin Conjugates

(2014) H. C. Besche et al.   EMBO JOURNAL

Cleaning up in the endoplasmic reticulum: ubiquitin in charge

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

The eIF2α kinases: their structures and functions

(2013) Neysan Donnelly et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Charcot–Marie–Tooth 1B caused by expansion of a familial myelin protein zero (MPZ) gene duplication

(2013) Marsha D. Speevak et al.   European Journal of Medical Genetics

Ubiquitinated Proteins Activate the Proteasomal ATPases by Binding to Usp14 or Uch37 Homologs

(2013) Andreas Peth et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

Structure of the 26S proteasome with ATP- S bound provides insights into the mechanism of nucleotide-dependent substrate translocation

(2013) P. Sledz et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Ubiquitin Code

(2012) David Komander et al.   Annual Review of Biochemistry

Phenotypic presentation of the Ser63Del MPZ mutation

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

Increased gene dosage of myelin protein zero causes Charcot-Marie-Tooth disease

(2011) Meiko Hashimoto Maeda et al.   ANNALS OF NEUROLOGY

Misfolded PrP impairs the UPS by interaction with the 20S proteasome and inhibition of substrate entry

(2011) Pelagia Deriziotis et al.   EMBO JOURNAL

P0S63del impedes the arrival of wild-type P0 glycoprotein to myelin in CMT1B mice

(2011) Pietro Fratta et al.   HUMAN MOLECULAR GENETICS

Quantitative and Integrative Proteome Analysis of Peripheral Nerve Myelin Identifies Novel Myelin Proteins and Candidate Neuropathy Loci

(2011) J. Patzig et al.   JOURNAL OF NEUROSCIENCE

Systematic and Quantitative Assessment of the Ubiquitin-Modified Proteome

(2011) Woong Kim et al.   MOLECULAR CELL

The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation

(2011) P. Walter et al.   SCIENCE

PLP/DM20 Expression and turnover in a transgenic mouse model of pelizaeus-merzbacher disease

(2010) Saadia A. Karim et al.   GLIA

Trimming of Ubiquitin Chains by Proteasome-associated Deubiquitinating Enzymes

(2010) Min Jae Lee et al.   MOLECULAR & CELLULAR PROTEOMICS

Transcription Factor Nrf1 Mediates the Proteasome Recovery Pathway after Proteasome Inhibition in Mammalian Cells

(2010) Senthil K. Radhakrishnan et al.   MOLECULAR CELL

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

(2010) Byung-Hoon Lee et al.   NATURE

Recognition and Processing of Ubiquitin-Protein Conjugates by the Proteasome

(2009) Daniel Finley   Annual Review of Biochemistry

Isolation of Mammalian 26S Proteasomes and p97/VCP Complexes Using the Ubiquitin-like Domain from HHR23B Reveals Novel Proteasome-Associated Proteins†

(2009) Henrike C. Besche et al.   BIOCHEMISTRY

Myelin under stress

(2009) Maurizio D'Antonio et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Ubiquitinated Proteins Activate the Proteasome by Binding to Usp14/Ubp6, which Causes 20S Gate Opening

(2009) Andreas Peth et al.   MOLECULAR CELL

Molecular mechanisms of inherited demyelinating neuropathies

(2008) Steven S. Scherer et al.   GLIA

The expression of tubulin polymerization promoting protein TPPP/p25α is developmentally regulated in cultured rat brain oligodendrocytes and affected by proteolytic stress

(2008) Olaf Goldbaum et al.   GLIA

Control of Cellular GADD34 Levels by the 26S Proteasome

(2008) M. H. Brush et al.   MOLECULAR AND CELLULAR BIOLOGY

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

(2008) Maria Pennuto et al.   NEURON