The Ubiquitin Proteasome System Is a Key Regulator of Pluripotent Stem Cell Survival and Motor Neuron Differentiation

Dynamic interplay between H-current and M-current controls motoneuron hyperexcitability in amyotrophic lateral sclerosis

(2019) Yossi Buskila et al.   Cell Death & Disease

The metastability of the proteome of spinal motor neurons underlies their selective vulnerability in ALS

(2019) Justin J. Yerbury et al.   NEUROSCIENCE LETTERS

SOD1A4Vaggregation alters ubiquitin homeostasis in a cell model of ALS

(2018) Natalie E. Farrawell et al.   JOURNAL OF CELL SCIENCE

Advances in therapy for spinal muscular atrophy: promises and challenges

(2018) Ewout J. N. Groen et al.   Nature Reviews Neurology

Targeting the 26S Proteasome To Protect Against Proteotoxic Diseases

(2018) Natura Myeku et al.   TRENDS IN MOLECULAR MEDICINE

Elimination of TDP-43 inclusions linked to amyotrophic lateral sclerosis by a misfolding-specific intrabody with dual proteolytic signals

(2018) Yoshitaka Tamaki et al.   Scientific Reports

Insights into the ubiquitin-proteasome system of human embryonic stem cells

(2018) Isabel Saez et al.   Scientific Reports

Generation and characterization of human induced pluripotent stem cell lines from a familial Alzheimer's disease PSEN1 A246E patient and a non-demented family member bearing wild-type PSEN1

(2018) Sonia Sanz Muñoz et al.   Stem Cell Research

Viral-free generation and characterization of a human induced pluripotent stem cell line from dermal fibroblasts

(2018) Martin Engel et al.   Stem Cell Research

Cytoscape StringApp: Network Analysis and Visualization of Proteomics Data

(2018) Nadezhda T. Doncheva et al.   JOURNAL OF PROTEOME RESEARCH

Impairments in Motor Neurons, Interneurons and Astrocytes Contribute to Hyperexcitability in ALS: Underlying Mechanisms and Paths to Therapy

(2017) Dzung Do-Ha et al.   MOLECULAR NEUROBIOLOGY

Spinal motor neuron protein supersaturation patterns are associated with inclusion body formation in ALS

(2017) Prajwal Ciryam et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Proteasome-independent polyubiquitin linkage regulates synapse scaffolding, efficacy, and plasticity

(2017) Qi Ma et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Potent and reversible lentiviral vector restriction in murine induced pluripotent stem cells

(2017) Franziska K. Geis et al.   Retrovirology

The unique spliceosome signature of human pluripotent stem cells is mediated by SNRPA1, SNRPD1, and PNN

(2017) Young-Dae Kim et al.   Stem Cell Research

Insulin and diet-induced changes in the ubiquitin-modified proteome of rat liver

(2017) Shilpa R. Nagarajan et al.   PLoS One

Common pitfalls of stem cell differentiation: a guide to improving protocols for neurodegenerative disease models and research

(2016) Martin Engel et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Walking the tightrope: proteostasis and neurodegenerative disease

(2016) Justin J. Yerbury et al.   JOURNAL OF NEUROCHEMISTRY

The STRING database in 2017: quality-controlled protein–protein association networks, made broadly accessible

(2016) Damian Szklarczyk et al.   NUCLEIC ACIDS RESEARCH

Unveiling the Structural and Dynamic Nature of the Ubiquitin Code

(2016) Paola Di Lello et al.   STRUCTURE

The Ball and Chain of Polyubiquitin Structures

(2016) Caterina Alfano et al.   TRENDS IN BIOCHEMICAL SCIENCES

CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia

(2016) Kelly L. Williams et al.   Nature Communications

Neuroprotective effects of apigenin against inflammation, neuronal excitability and apoptosis in an induced pluripotent stem cell model of Alzheimer’s disease

(2016) Rachelle Balez et al.   Scientific Reports

InteractiVenn: a web-based tool for the analysis of sets through Venn diagrams

(2015) Henry Heberle et al.   BMC BIOINFORMATICS

Evolution of Proteasome Regulators in Eukaryotes

(2015) Philippe Fort et al.   Genome Biology and Evolution

Ubiquitin–Synaptobrevin Fusion Protein Causes Degeneration of Presynaptic Motor Terminals in Mice

(2015) Yun Liu et al.   JOURNAL OF NEUROSCIENCE

SOD1 protein aggregates stimulate macropinocytosis in neurons to facilitate their propagation

(2015) Rafaa Zeineddine et al.   Molecular Neurodegeneration

Generation and expansion of highly pure motor neuron progenitors from human pluripotent stem cells

(2015) Zhong-Wei Du et al.   Nature Communications

Ubiquitin-activating enzyme E1 inhibitor PYR41 attenuates angiotensin II-induced activation of dendritic cells via the IκBa/NF-κB and MKP1/ERK/STAT1 pathways

(2014) Chen Chen et al.   IMMUNOLOGY

High-efficiency motor neuron differentiation from human pluripotent stem cells and the function of Islet-1

(2014) Qiuhao Qu et al.   Nature Communications

Protein quality control and elimination of protein waste: The role of the ubiquitin–proteasome system

(2013) Ingo Amm et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Clinical and neuropathological features of X-linked spinal muscular atrophy (SMAX2) associated with a novel mutation in the UBA1 gene

(2013) Nomazulu Dlamini et al.   NEUROMUSCULAR DISORDERS

Spatial organization of ubiquitin ligase pathways orchestrates neuronal connectivity

(2013) Tomoko Yamada et al.   TRENDS IN NEUROSCIENCES

Breaking It Down: The Ubiquitin Proteasome System in Neuronal Morphogenesis

(2013) Andrew M. Hamilton et al.   NEURAL PLASTICITY

Unravelling the enigma of selective vulnerability in neurodegeneration: motor neurons resistant to degeneration in ALS show distinct gene expression characteristics and decreased susceptibility to excitotoxicity

(2012) Alice Brockington et al.   ACTA NEUROPATHOLOGICA

The Ubiquitin Code

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

Regulation of Pluripotency and Cellular Reprogramming by the Ubiquitin-Proteasome System

(2012) Shannon M. Buckley et al.   Cell Stem Cell

Spliceosome integrity is defective in the motor neuron diseases ALS and SMA

(2012) Hitomi Tsuiji et al.   EMBO Molecular Medicine

Induced pluripotent stem cells as tools for disease modelling and drug discovery in Alzheimer’s disease

(2012) Lezanne Ooi et al.   JOURNAL OF NEURAL TRANSMISSION

Increased proteasome activity in human embryonic stem cells is regulated by PSMD11

(2012) David Vilchez et al.   NATURE

Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability

(2012) B. Bilican et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

SQSTM1 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2011) Faisal Fecto   ARCHIVES OF NEUROLOGY

Andromeda: A Peptide Search Engine Integrated into the MaxQuant Environment

(2011) Jürgen Cox et al.   JOURNAL OF PROTEOME RESEARCH

The quantitative proteomes of human-induced pluripotent stem cells and embryonic stem cells

(2011) J. Munoz et al.   Molecular Systems Biology

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

An Expanded Oct4 Interaction Network: Implications for Stem Cell Biology, Development, and Disease

(2010) Mercedes Pardo et al.   Cell Stem Cell

Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

(2010) Janel O. Johnson et al.   NEURON

The ubiquitin proteasome system in neuropathology

(2009) Norman L. Lehman   ACTA NEUROPATHOLOGICA

A global view of protein expression in human cells, tissues, and organs

(2009) Fredrik Pontén et al.   Molecular Systems Biology

Systems-level dynamic analyses of fate change in murine embryonic stem cells

(2009) Rong Lu et al.   NATURE

Rare Missense and Synonymous Variants in UBE1 Are Associated with X-Linked Infantile Spinal Muscular Atrophy

(2008) Juliane Ramser et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Dysfunction of the ubiquitin-proteasome system in multiple disease conditions: therapeutic approaches

(2008) Subhankar Paul   BIOESSAYS

Induced Pluripotent Stem Cells Generated from Patients with ALS Can Be Differentiated into Motor Neurons

(2008) J. T. Dimos et al.   SCIENCE