[HTML][HTML] Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is an idiopathic, fatal neurodegenerative disease of the
human motor system. In this Seminar, we summarise current concepts about the origin of the …
human motor system. In this Seminar, we summarise current concepts about the origin of the …
Energy metabolism in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is characterised by the progressive degeneration of
upper and lower motor neurons. Besides motor neuron degeneration, ALS is associated …
upper and lower motor neurons. Besides motor neuron degeneration, ALS is associated …
Motor unit and neuromuscular junction remodeling with aging
MR Deschenes - Current aging science, 2011 - ingentaconnect.com
The neuromuscular system is one of the largest and most vital organ systems of the body.
The function and mass of the neuromuscular system gradually deteriorate during the natural …
The function and mass of the neuromuscular system gradually deteriorate during the natural …
Optimised and Rapid Pre-clinical Screening in the SOD1G93A Transgenic Mouse Model of Amyotrophic Lateral Sclerosis (ALS)
The human SOD1G93A transgenic mouse has been used extensively since its development
in 1994 as a model for amyotrophic lateral sclerosis (ALS). In that time, a great many insights …
in 1994 as a model for amyotrophic lateral sclerosis (ALS). In that time, a great many insights …
Abnormalities of satellite cells function in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is characterized by progressive denervation leading to
muscle atrophy prevented, during the early phase, by compensatory reinnervation. Little is …
muscle atrophy prevented, during the early phase, by compensatory reinnervation. Little is …
Motor neuron trophic factors: therapeutic use in ALS?
TW Gould, RW Oppenheim - Brain research reviews, 2011 - Elsevier
The modest effects of neurotrophic factor (NTF) treatment on lifespan in both animal models
and clinical studies of Amyotropic Lateral Sclerosis (ALS) may result from any one or …
and clinical studies of Amyotropic Lateral Sclerosis (ALS) may result from any one or …
Neuroprotection by gene therapy targeting mutant SOD1 in individual pools of motor neurons does not translate into therapeutic benefit in fALS mice
C Towne, V Setola, BL Schneider, P Aebischer - Molecular Therapy, 2011 - cell.com
A major challenge in neurological gene therapy is delivery of the transgene to sufficient cell
numbers in an atraumatic manner. This is particularly difficult for motor neuron (MN) …
numbers in an atraumatic manner. This is particularly difficult for motor neuron (MN) …
Altered expression of myogenic regulatory factors in the mouse model of amyotrophic lateral sclerosis
R Manzano, JM Toivonen, S Oliván, AC Calvo… - Neurodegenerative …, 2011 - karger.com
Background: In the superoxide dismutase 1 (SOD1)-G93A mouse model of amyotrophic
lateral sclerosis (ALS), skeletal muscle is a key target of mutant SOD1 toxicity. However, the …
lateral sclerosis (ALS), skeletal muscle is a key target of mutant SOD1 toxicity. However, the …
Respiration and ROS production in brain and spinal cord mitochondria of transgenic rats with mutant G93a Cu/Zn-superoxide dismutase gene
A Panov, N Kubalik, N Zinchenko, R Hemendinger… - Neurobiology of …, 2011 - Elsevier
Mitochondrial dysfunction is involved in the pathogenesis of motor neuron degeneration in
the G93A mutant transgenic (tgmSOD1) animal model of ALS. However, it is unknown …
the G93A mutant transgenic (tgmSOD1) animal model of ALS. However, it is unknown …
Comparison of muscle ultrastructure in myasthenia gravis with anti-MuSK and anti-AChR antibodies
G Cenacchi, P Valentina, F Marina, P Elena… - Journal of …, 2011 - Springer
Patients with myasthenia gravis (MG) with antibodies to muscle-specific receptor tyrosine
kinase (MuSK) differ from acetylcholine receptor (AChR)-positive MG patients, as they …
kinase (MuSK) differ from acetylcholine receptor (AChR)-positive MG patients, as they …