Functional skeletal muscle model derived from SOD1-mutant ALS patient iPSCs recapitulates hallmarks of disease progression

Diagnostic Challenge and Neuromuscular Junction Contribution to ALS Pathogenesis

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Structural Properties and Interaction Partners of Familial ALS-Associated SOD1 Mutants

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Far positioned ALS associated mutants of Cu/Zn SOD forms partially metallated, destabilized misfolding intermediates

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FUS-mediated regulation of acetylcholine receptor transcription at neuromuscular junctions is compromised in amyotrophic lateral sclerosis

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

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Metabolic Changes Associated With Muscle Expression of SOD1G93A

(2018) Gabriella Dobrowolny et al.   Frontiers in Physiology

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

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Dexamethasone Treatment at the Myoblast Stage Enhanced C2C12 Myocyte Differentiation

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Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis

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The Role of Skeletal Muscle in Amyotrophic Lateral Sclerosis

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Elimination Rate of Serum Lactate is Correlated with Amyotrophic Lateral Sclerosis Progression

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Generation of human muscle fibers and satellite-like cells from human pluripotent stem cells in vitro

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Acetylcholine receptors from human muscle as pharmacological targets for ALS therapy

(2016) Eleonora Palma 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

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Skeletal Muscle Remodelling as a Function of Disease Progression in Amyotrophic Lateral Sclerosis

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Skeletal Muscle Satellite Cells, Mitochondria, and MicroRNAs: Their Involvement in the Pathogenesis of ALS

(2016) Stavroula Tsitkanou et al.   Frontiers in Physiology

Muscle as a “Mediator” of Systemic Metabolism

(2015) Kedryn K. Baskin et al.   Cell Metabolism

A novel in vitro model of sarcopenia using BubR1 hypomorphic C2C12 myoblasts

(2015) Takateru Nozaki et al.   CYTOTECHNOLOGY

A metabolic switch toward lipid use in glycolytic muscle is an early pathologic event in a mouse model of amyotrophic lateral sclerosis

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A retrospective review of the progress in amyotrophic lateral sclerosis drug discovery over the last decade and a look at the latest strategies

(2015) Abigail DeLoach et al.   Expert Opinion on Drug Discovery

Role of mitochondria in mutant SOD1 linked amyotrophic lateral sclerosis

(2014) Wenzhi Tan et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Mechanistic investigation of adult myotube response to exercise and drug treatment in vitro using a multiplexed functional assay system

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Skeletal Muscle Satellite Cells in Amyotrophic Lateral Sclerosis

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Amyotrophic Lateral Sclerosis and Skeletal Muscle: An Update

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

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Defective Mitochondrial Dynamics Is an Early Event in Skeletal Muscle of an Amyotrophic Lateral Sclerosis Mouse Model

(2013) Guo Luo et al.   PLoS One

Tissue-Specific Stem Cells: Lessons from the Skeletal Muscle Satellite Cell

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Abnormalities of satellite cells function in amyotrophic lateral sclerosis

(2011) Pierre-François Pradat et al.   Amyotrophic Lateral Sclerosis

Hind limb muscle atrophy precedes cerebral neuronal degeneration in G93A-SOD1 mouse model of amyotrophic lateral sclerosis: A longitudinal MRI study

(2011) Stefania Marcuzzo et al.   EXPERIMENTAL NEUROLOGY

Skeletal muscle-restricted expression of human SOD1 causes motor neuron degeneration in transgenic mice

(2010) Margaret Wong et al.   HUMAN MOLECULAR GENETICS

Measurement of Contractile Stress Generated by Cultured Rat Muscle on Silicon Cantilevers for Toxin Detection and Muscle Performance Enhancement

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Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated Toxicity

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Regulation of oxidative phosphorylation, the mitochondrial membrane potential, and their role in human disease

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Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

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