ArticleNeurogenetics2010
Reduced levels of survival motor neuron protein leads to aberrant motoneuron growth in a Xenopus model of muscular atrophy.
Article in Neurogenetics, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 26 citations in OpenAlex.
- Local Protein Synthesis at Synapses: A Driver for Synapse Diversification.Journal of neurochemistry · 2025Review
- Functional characterization of SMN evolution in mouse models of SMA.Scientific reports · 2019Article
- mRNP assembly, axonal transport, and local translation in neurodegenerative diseases.Brain research · 2018Review
- Hermes Regulates Axon Sorting in the Optic Tract by Post-Trancriptional Regulation of Neuropilin 1.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2016Article
- Research proceedings on amphibian model organisms.Dong wu xue yan jiu = Zoological research · 2016Review
- Deficiency of the Survival of Motor Neuron Protein Impairs mRNA Localization and Local Translation in the Growth Cone of Motor Neurons.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2016Article
- Decay in survival motor neuron and plastin 3 levels during differentiation of iPSC-derived human motor neurons.Scientific reports · 2015Article
- Comparison of T7E1 and surveyor mismatch cleavage assays to detect mutations triggered by engineered nucleases.G3 (Bethesda, Md.) · 2015Article
- Article
- Temporal requirement for SMN in motoneuron development.Human molecular genetics · 2013Article
- RNA-binding proteins and translational regulation in axons and growth cones.Frontiers in neuroscience · 2013Article
- Spinal muscular atrophy: the role of SMN in axonal mRNA regulation.Brain research · 2012Review
- Inhibition of apoptosis blocks human motor neuron cell death in a stem cell model of spinal muscular atrophy.PloS one · 2012Article
- Annotation of the transcriptome from Taenia pisiformis and its comparative analysis with three Taeniidae species.PloS one · 2012Article
- Generation and Characterization of a genetic zebrafish model of SMA carrying the human SMN2 gene.Molecular neurodegeneration · 2011Article
Corrections and comments
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Authors and funding
8 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Spinal muscular atrophy (SMA) is a neurodegenerative disease characterized by motor neuron loss and skeletal muscle atrophy. The loss of function of the smn1 gene, the main supplier of survival motor neuron protein (SMN) protein in human, leads to reduced levels of SMN and eventually to SMA. Here, we ask if the amphibian Xenopus tropicalis can be a good model system to study SMA. Inhibition of the production of SMN using antisense morpholinos leads to caudal muscular atrophy in tadpoles. Of note, early developmental patterning of muscles and motor neurons is unaffected in this system as well as acetylcholine receptors clustering. Muscular atrophy seems to rather result from aberrant pathfinding and growth arrest and/or shortening of motor axons. This event occurs in the absence of neuronal cell bodies apoptosis, a process comparable to that of amyotrophic lateral sclerosis. Xenopus tropicalis is revealed as a complementary animal model for the study of SMA.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.