Amyotrophic Lateral Sclerosis and Skeletal Muscle: An Update

Protein misfolding, mitochondrial dysfunction and muscle loss are not directly dependent on soluble and aggregation state of mSOD1 protein in skeletal muscle of ALS

(2012) Rochelle Wei et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Elevated PGC-1α Activity Sustains Mitochondrial Biogenesis and Muscle Function without Extending Survival in a Mouse Model of Inherited ALS

(2012) Sandrine Da Cruz et al.   Cell Metabolism

Altered in vitro Proliferation of Mouse SOD1-G93A Skeletal Muscle Satellite Cells

(2012) Raquel Manzano et al.   Neurodegenerative Diseases

The BH4 domain of Bcl-XL rescues astrocyte degeneration in amyotrophic lateral sclerosis by modulating intracellular calcium signals

(2011) Francesca Martorana et al.   HUMAN MOLECULAR GENETICS

Muscle cells and motoneurons differentially remove mutant SOD1 causing familial amyotrophic lateral sclerosis

(2011) Elisa Onesto et al.   JOURNAL OF NEUROCHEMISTRY

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

(2011) Han-Xiang Deng et al.   NATURE

Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

(2011) Amanda M Haidet-Phillips et al.   NATURE BIOTECHNOLOGY

Altered Expression of Myogenic Regulatory Factors in the Mouse Model of Amyotrophic Lateral Sclerosis

(2011) Raquel Manzano et al.   Neurodegenerative Diseases

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

Phenotypically aberrant astrocytes that promote motoneuron damage in a model of inherited amyotrophic lateral sclerosis

(2011) P. Diaz-Amarilla et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo

(2011) S. T. Papadeas et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Quantity and Activation of Myofiber-Associated Satellite Cells in a Mouse Model of Amyotrophic Lateral Sclerosis

(2011) Raquel Manzano et al.   Stem Cell Reviews and Reports

TDP-43 in skeletal muscle of patients affected with amyotrophic lateral sclerosis

(2010) Gianni Sorarú et al.   Amyotrophic Lateral Sclerosis

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

(2010) Margaret Wong et al.   HUMAN MOLECULAR GENETICS

Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice

(2010) L. Wang et al.   HUMAN MOLECULAR GENETICS

Repairing skeletal muscle: regenerative potential of skeletal muscle stem cells

(2010) Francesco Saverio Tedesco et al.   JOURNAL OF CLINICAL INVESTIGATION

Abnormal TDP-43 and FUS proteins in muscles of sporadic IBM: similarities in a TARDBP-linked ALS patient

(2010) A. Hernandez Lain et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Oxidative stress in ALS: Key role in motor neuron injury and therapeutic target

(2009) Siân C. Barber et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

Focal degeneration of astrocytes in amyotrophic lateral sclerosis

(2008) D Rossi et al.   CELL DEATH AND DIFFERENTIATION

Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated Toxicity

(2008) Gabriella Dobrowolny et al.   Cell Metabolism

Non-Cell-Autonomous Effect of Human SOD1G37R Astrocytes on Motor Neurons Derived from Human Embryonic Stem Cells

(2008) Maria C.N. Marchetto et al.   Cell Stem Cell

Human Embryonic Stem Cell-Derived Motor Neurons Are Sensitive to the Toxic Effect of Glial Cells Carrying an ALS-Causing Mutation

(2008) Francesco Paolo Di Giorgio et al.   Cell Stem Cell

Mitochondrial Dysfunction in SOD1G93A-Bearing Astrocytes Promotes Motor Neuron Degeneration: Prevention by Mitochondrial-Targeted Antioxidants

(2008) P. Cassina et al.   JOURNAL OF NEUROSCIENCE

Preferential motor unit loss in the SOD1G93Atransgenic mouse model of amyotrophic lateral sclerosis

(2008) J. Hegedus et al.   JOURNAL OF PHYSIOLOGY-LONDON

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

(2008) Koji Yamanaka et al.   NATURE NEUROSCIENCE