Chronic Treatment with the AMPK Agonist AICAR Prevents Skeletal Muscle Pathology but Fails to Improve Clinical Outcome in a Mouse Model of Severe Spinal Muscular Atrophy

Spinal muscular atrophy (SMA) is a genetic neuromuscular disorder characterized by spinal and brainstem motor neuron (MN) loss and skeletal muscle paralysis. Currently, there is no effective treatment other than supportive care to ameliorate the quality of life of patients with SMA. Some studies hav...

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Veröffentlicht in:	Neurotherapeutics 2016-01, Vol.13 (1), p.198-216
Hauptverfasser:	Cerveró, Clàudia, Montull, Neus, Tarabal, Olga, Piedrafita, Lídia, Esquerda, Josep E., Calderó, Jordi
Format:	Artikel
Sprache:	eng
Schlagworte:	Aminoimidazole Carboxamide - analogs & derivatives Aminoimidazole Carboxamide - therapeutic use AMP-Activated Protein Kinases - antagonists & inhibitors Animals Atrophy Biomedical and Life Sciences Biomedicine Disease Models, Animal Exercise Female Kinases Male Mice Mice, Knockout Muscle, Skeletal - drug effects Muscle, Skeletal - pathology Muscular Atrophy, Spinal - drug therapy Muscular Atrophy, Spinal - pathology Musculoskeletal system Neurobiology Neurology Neurosciences Neurosurgery Original Original Article Palliative care Paralysis Proteins Quality of life Ribonucleotides - therapeutic use Spinal cord Spinal Cord - drug effects Spinal Cord - pathology Treatment Outcome
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