Bioenergetic status modulates motor neuron vulnerability and pathogenesis in a zebrafish model of spinal muscular atrophy

Degeneration and loss of lower motor neurons is the major pathological hallmark of spinal muscular atrophy (SMA), resulting from low levels of ubiquitously-expressed survival motor neuron (SMN) protein. One remarkable, yet unresolved, feature of SMA is that not all motor neurons are equally affected...

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Veröffentlicht in:	PLoS genetics 2017-04, Vol.13 (4), p.e1006744-e1006744
Hauptverfasser:	Boyd, Penelope J, Tu, Wen-Yo, Shorrock, Hannah K, Groen, Ewout J N, Carter, Roderick N, Powis, Rachael A, Thomson, Sophie R, Thomson, Derek, Graham, Laura C, Motyl, Anna A L, Wishart, Thomas M, Highley, J Robin, Morton, Nicholas M, Becker, Thomas, Becker, Catherina G, Heath, Paul R, Gillingwater, Thomas H
Format:	Artikel
Sprache:	eng
Schlagworte:	Adenosine Triphosphate - metabolism Amyotrophic lateral sclerosis Animals Axons - metabolism Axons - pathology Bioenergetics Biology and Life Sciences Cell survival Comparative analysis Degeneration Development and progression Disease Models, Animal Disease resistance Disease Susceptibility Energy Metabolism Gene expression Gene Expression Regulation, Developmental Genetic aspects Humans Medicine and Health Sciences Mice Mitochondria Mitochondria - metabolism Motor neurons Motor Neurons - drug effects Motor Neurons - metabolism Motor task performance Muscle, Skeletal - metabolism Muscle, Skeletal - pathology Muscles Muscular Atrophy, Spinal - genetics Muscular Atrophy, Spinal - metabolism Muscular Atrophy, Spinal - physiopathology Neuromuscular diseases Neurosciences Phosphoglycerate kinase Phosphoglycerate Kinase - antagonists & inhibitors Phosphoglycerate Kinase - genetics Phosphoglycerate kinase 1 Physiological aspects Prazosin - administration & dosage Prazosin - analogs & derivatives Research and Analysis Methods Rodents Skeletal muscle SMN protein Spinal Cord - growth & development Spinal Cord - metabolism Spinal Cord - pathology Spinal muscular atrophy Survival of Motor Neuron 1 Protein - genetics Survival of Motor Neuron 1 Protein - metabolism Vulnerability Zebrafish Zebrafish - genetics Zebrafish - growth & development
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