Modelling FUS Mislocalisation in an In Vitro Model of Innervated Human Muscle

Nuclear trafficking in amyotrophic lateral sclerosis and frontotemporal lobar degeneration

(2016) Sonja Prpar Mihevc et al.   BRAIN

The Role of Skeletal Muscle in Amyotrophic Lateral Sclerosis

(2016) Jean-Philippe Loeffler et al.   BRAIN PATHOLOGY

Oligodendrocytes contribute to motor neuron death in ALS via SOD1-dependent mechanism

(2016) Laura Ferraiuolo et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Differentiation of control and ALS mutant human iPSCs into functional skeletal muscle cells, a tool for the study of neuromuscolar diseases

(2016) Jessica Lenzi et al.   Stem Cell Research

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

(2016) Aarti Sharma et al.   Nature Communications

Verification of the Cross Immunoreactivity of A60, a Mouse Monoclonal Antibody against Neuronal Nuclear Protein

(2016) Shanping Mao et al.   Frontiers in Neuroanatomy

TDP-43 aggregation mirrors TDP-43 knockdown, affecting the expression levels of a common set of proteins

(2016) S. Prpar Mihevc et al.   Scientific Reports

siRNA delivery into cultured primary human myoblasts - optimization of electroporation parameters and theoretical analysis

(2015) Jasna Lojk et al.   BIOELECTROMAGNETICS

The function of RNA-binding proteins at the synapse: implications for neurodegeneration

(2015) Chantelle F. Sephton et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

ALS mutant FUS proteins are recruited into stress granules in induced pluripotent stem cell-derived motoneurons

(2015) J. Lenzi et al.   Disease Models & Mechanisms

Phosphorylation of C-terminal tyrosine residue 526 in FUS impairs its nuclear import

(2015) S. Darovic et al.   JOURNAL OF CELL SCIENCE

Emerging mechanisms of molecular pathology in ALS

(2015) Owen M. Peters et al.   JOURNAL OF CLINICAL INVESTIGATION

Cross-disease comparison of amyotrophic lateral sclerosis and spinal muscular atrophy reveals conservation of selective vulnerability but differential neuromuscular junction pathology

(2015) Laura H. Comley et al.   JOURNAL OF COMPARATIVE NEUROLOGY

Early and Persistent Abnormal Decoding by Glial Cells at the Neuromuscular Junction in an ALS Model

(2015) D. Arbour et al.   JOURNAL OF NEUROSCIENCE

Calcium dysregulation links ALS defective proteins and motor neuron selective vulnerability

(2015) Sónia S. Leal et al.   Frontiers in Cellular Neuroscience

New Insights into the Mechanisms of Gene Electrotransfer – Experimental and Theoretical Analysis

(2015) Mojca Pavlin et al.   Scientific Reports

Human TDP-43 and FUS selectively affect motor neuron maturation and survival in a murine cell model of ALS by non-cell-autonomous mechanisms

(2015) Nicole Wächter et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Dexamethasone Produces Dose-Dependent Inhibition of Sugammadex Reversal in In Vitro Innervated Primary Human Muscle Cells

(2014) Katja Rezonja et al.   ANESTHESIA AND ANALGESIA

The ALS gene FUS regulates synaptic transmission at the Drosophila neuromuscular junction

(2014) James B. Machamer et al.   HUMAN MOLECULAR GENETICS

Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species

(2014) E. L. Scotter et al.   JOURNAL OF CELL SCIENCE

Necroptosis Drives Motor Neuron Death in Models of Both Sporadic and Familial ALS

(2014) Diane B. Re et al.   NEURON

Skeletal Muscle Satellite Cells in Amyotrophic Lateral Sclerosis

(2014) Annarita Scaramozza et al.   ULTRASTRUCTURAL PATHOLOGY

Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis

(2013) Thomas Philips et al.   BRAIN

Loss and gain of FUS function impair neuromuscular synaptic transmission in a genetic model of ALS

(2013) Gary A.B. Armstrong et al.   HUMAN MOLECULAR GENETICS

Stress granules as crucibles of ALS pathogenesis

(2013) Yun R. Li et al.   JOURNAL OF CELL BIOLOGY

Defects in Synapse Structure and Function Precede Motor Neuron Degeneration in Drosophila Models of FUS-Related ALS

(2013) M. Shahidullah et al.   JOURNAL OF NEUROSCIENCE

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

Opposing effects of dexamethasone, agrin and sugammadex on functional innervation and constitutive secretion of IL-6 in in vitro innervated primary human muscle cells

(2013) K. Rezonja et al.   NEUROSCIENCE LETTERS

Early Changes of Neuromuscular Transmission in the SOD1(G93A) Mice Model of ALS Start Long before Motor Symptoms Onset

(2013) Mariana C. Rocha et al.   PLoS One

Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy

(2013) A. Serio et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Parkin Ubiquitinates Tar-DNA Binding Protein-43 (TDP-43) and Promotes Its Cytosolic Accumulation via Interaction with Histone Deacetylase 6 (HDAC6)

(2012) Michaeline L. Hebron et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs

(2012) Clotilde Lagier-Tourenne et al.   NATURE NEUROSCIENCE

Shared Resistance to Aging and ALS in Neuromuscular Junctions of Specific Muscles

(2012) Gregorio Valdez et al.   PLoS One

Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain

(2012) Boris Rogelj et al.   Scientific Reports

p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS

(2011) Safa Al-Sarraj et al.   ACTA NEUROPATHOLOGICA

Neuron–glia interactions: the roles of Schwann cells in neuromuscular synapse formation and function

(2011) Yoshie Sugiura et al.   BIOSCIENCE REPORTS

Nuclear import impairment causes cytoplasmic trans-activation response DNA-binding protein accumulation and is associated with frontotemporal lobar degeneration

(2010) Agnes L. Nishimura et al.   BRAIN

The Role of Electrophoresis in Gene Electrotransfer

(2010) M. Pavlin et al.   JOURNAL OF MEMBRANE BIOLOGY

Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6

(2009) C. Vance et al.   SCIENCE

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

(2008) Koji Yamanaka et al.   NATURE NEUROSCIENCE

Neural agrin controls maturation of the excitation-contraction coupling mechanism in human myotubes developing in vitro

(2007) Elena Bandi et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY