Human motor units in microfluidic devices are impaired by FUS mutations and improved by HDAC6 inhibition

HDAC6 inhibition restores TDP‐43 pathology and axonal transport defects in human motor neurons with TARDBP mutations

(2021) Raheem Fazal et al.   EMBO JOURNAL

HDAC6 inhibitors: Translating genetic and molecular insights into a therapy for axonal CMT

(2020) Elisabeth Rossaert et al.   BRAIN RESEARCH

HDAC6 regulates microtubule stability and clustering of AChRs at neuromuscular junctions

(2020) Alexis Osseni et al.   JOURNAL OF CELL BIOLOGY

A 3D culture model of innervated human skeletal muscle enables studies of the adult neuromuscular junction

(2019) Mohsen Afshar Bakooshli et al.   eLife

Widespread FUS mislocalization is a molecular hallmark of amyotrophic lateral sclerosis

(2019) Giulia E Tyzack et al.   BRAIN

Aberrant axon branching via Fos-B dysregulation in FUS-ALS motor neurons

(2019) Tetsuya Akiyama et al.   EBioMedicine

Restoration of histone acetylation ameliorates disease and metabolic abnormalities in a FUS mouse model

(2019) Elisabeth Rossaert et al.   Acta Neuropathologica Communications

Axonal transport defects and neurodegeneration: Molecular mechanisms and therapeutic implications

(2019) Wenting Guo et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Differentiation but not ALS mutations in FUS rewires motor neuron metabolism

(2019) Tijs Vandoorne et al.   Nature Communications

A customizable microfluidic platform for medium-throughput modeling of neuromuscular circuits

(2019) Jessica Bellmann et al.   BIOMATERIALS

Aging affects the in vivo regenerative potential of human mesoangioblasts

(2018) Alessio Rotini et al.   AGING CELL

Stem cell derived phenotypic human neuromuscular junction model for dose response evaluation of therapeutics

(2018) Navaneetha Santhanam et al.   BIOMATERIALS

HDAC6 is a therapeutic target in mutant GARS-induced Charcot-Marie-Tooth disease

(2018) Veronick Benoy et al.   BRAIN

Fundamental Molecules and Mechanisms for Forming and Maintaining Neuromuscular Synapses

(2018) Steven Burden et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Inhibition of histone deacetylase 6 (HDAC6) protects against vincristine-induced peripheral neuropathies and inhibits tumor growth

(2018) Lawrence Van Helleputte et al.   NEUROBIOLOGY OF DISEASE

Microfluidics of Small-Population Neurons Allows for a Precise Quantification of the Peripheral Axonal Growth State

(2018) Georg Jocher et al.   Frontiers in Cellular Neuroscience

Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation

(2018) Marcel Naumann et al.   Nature Communications

Engineered 3D vascular and neuronal networks in a microfluidic platform

(2018) Tatsuya Osaki et al.   Scientific Reports

Neurturin is a PGC-1α1-controlled myokine that promotes motor neuron recruitment and neuromuscular junction formation

(2018) Richard Mills et al.   Molecular Metabolism

Modeling sporadic ALS in iPSC-derived motor neurons identifies a potential therapeutic agent

(2018) Koki Fujimori et al.   NATURE MEDICINE

Mutant FUS causes DNA ligation defects to inhibit oxidative damage repair in Amyotrophic Lateral Sclerosis

(2018) Haibo Wang et al.   Nature Communications

HDAC6 as a potential therapeutic target for peripheral nerve disorders

(2018) Robert Prior et al.   EXPERT OPINION ON THERAPEUTIC TARGETS

Dynamic neuromuscular remodeling precedes motor-unit loss in a mouse model of ALS

(2018) Éric Martineau et al.   eLife

Axon-Seq Decodes the Motor Axon Transcriptome and Its Modulation in Response to ALS

(2018) Jik Nijssen et al.   Stem Cell Reports

Microphysiological 3D model of amyotrophic lateral sclerosis (ALS) from human iPS-derived muscle cells and optogenetic motor neurons

(2018) Tatsuya Osaki et al.   Science Advances

Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic mice

(2017) Eva So et al.   HUMAN MOLECULAR GENETICS

HDAC6 inhibition effectively reverses chemotherapy-induced peripheral neuropathy

(2017) Karen Krukowski et al.   PAIN

HDAC6 inhibition reverses axonal transport defects in motor neurons derived from FUS-ALS patients

(2017) Wenting Guo et al.   Nature Communications

Cellular and Molecular Anatomy of the Human Neuromuscular Junction

(2017) Ross A. Jones et al.   Cell Reports

Length-dependent axo-terminal degeneration at the neuromuscular synapses of type II muscle in SOD1 mice

(2016) C. Tallon et al.   NEUROSCIENCE

Development of Improved HDAC6 Inhibitors as Pharmacological Therapy for Axonal Charcot–Marie–Tooth Disease

(2016) Veronick Benoy et al.   Neurotherapeutics

Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43

(2015) Adam K. Walker et al.   ACTA NEUROPATHOLOGICA

Combination of agrin and laminin increase acetylcholine receptor clustering and enhance functional neuromuscular junction formationIn vitro

(2015) Bill G.X. Zhang et al.   Developmental Neurobiology

A compartmentalized microfluidic neuromuscular co-culture system reveals spatial aspects of GDNF functions

(2015) E. E. Zahavi et al.   JOURNAL OF CELL SCIENCE

Formation and characterisation of neuromuscular junctions between hiPSC derived motoneurons and myotubes

(2015) M. Demestre et al.   Stem Cell Research

Generation of Functional Neuromuscular Junctions from Human Pluripotent Stem Cell Lines

(2015) Katja A. Puttonen et al.   Frontiers in Cellular Neuroscience

Combinatorial analysis of developmental cues efficiently converts human pluripotent stem cells into multiple neuronal subtypes

(2014) Yves Maury et al.   NATURE BIOTECHNOLOGY

Microfluidic primary culture model of the lower motor neuron–neuromuscular junction circuit

(2013) Katherine A. Southam et al.   JOURNAL OF NEUROSCIENCE METHODS

The changing scene of amyotrophic lateral sclerosis

(2013) Wim Robberecht et al.   NATURE REVIEWS NEUROSCIENCE

Questions regarding the predictive value of one evolved complex adaptive system for a second: Exemplified by the SOD1 mouse

(2013) Ray Greek et al.   PROGRESS IN BIOPHYSICS & MOLECULAR BIOLOGY

Functional Neuromuscular Junctions Formed by Embryonic Stem Cell-Derived Motor Neurons

(2012) Joy A. Umbach et al.   PLoS One

New perspectives on neuronal development via microfluidic environments

(2012) Larry J. Millet et al.   TRENDS IN NEUROSCIENCES

Neuromuscular junction formation between human stem cell-derived motoneurons and human skeletal muscle in a defined system

(2011) Xiufang Guo et al.   BIOMATERIALS

HDAC6 inhibitors reverse axonal loss in a mouse model of mutant HSPB1–induced Charcot-Marie-Tooth disease

(2011) Constantin d'Ydewalle et al.   NATURE MEDICINE

The “Dying-Back” Phenomenon of Motor Neurons in ALS

(2010) Michal Dadon-Nachum et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Diffusion tensor imaging reveals regional differences in the cervical spinal cord in amyotrophic lateral sclerosis

(2010) Govind Nair et al.   NEUROIMAGE

Review: Neuromuscular synaptic vulnerability in motor neurone disease: amyotrophic lateral sclerosis and spinal muscular atrophy

(2010) L. M. Murray et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY