ArticleHuman molecular genetics2015
Motoneuron development influences dorsal root ganglia survival and Schwann cell development in a vertebrate model of spinal muscular atrophy.
Article in Human molecular genetics, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 27 citations in OpenAlex.
- Longitudinal spinal cord MRI in adult 5q-SMA: biomarker and pathophysiological insights.Journal of neurology · 2026Article
- INHAND: Non-proliferative and proliferative lesions of fish - Chapter 8. Fish nervous system.Journal of toxicologic pathology · 2026Article
- In utero therapy for spinal muscular atrophy: closer to clinical translation.Brain : a journal of neurology · 2025Review
- Modeling Spinal Muscular Atrophy in Zebrafish: Current Advances and Future Perspectives.International journal of molecular sciences · 2024Review
- Fluorescence Correlation Spectroscopy Reveals Survival Motor Neuron Oligomerization but No Active Transport in Motor Axons of a Zebrafish Model for Spinal Muscular Atrophy.Frontiers in cell and developmental biology · 2021Article
- Molecular Mechanisms Underlying Sensory-Motor Circuit Dysfunction in SMA.Frontiers in molecular neuroscience · 2019Article
- mRNP assembly, axonal transport, and local translation in neurodegenerative diseases.Brain research · 2018Review
- HuD and the Survival Motor Neuron Protein Interact in Motoneurons and Are Essential for Motoneuron Development, Function, and mRNA Regulation.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2017Article
- SMN regulation in SMA and in response to stress: new paradigms and therapeutic possibilities.Human genetics · 2017Review
- Therapeutic strategies for spinal muscular atrophy: SMN and beyond.Disease models & mechanisms · 2017Review
- The Survival of Motor Neuron Protein Acts as a Molecular Chaperone for mRNP Assembly.Cell reports · 2017Article
- Dysregulation of mRNA Localization and Translation in Genetic Disease.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2016Review
- Altered Levels of MicroRNA-9, -206, and -132 in Spinal Muscular Atrophy and Their Response to Antisense Oligonucleotide Therapy.Molecular therapy. Nucleic acids · 2016Article
- Transcriptional enhancement of Smn levels in motoneurons is crucial for proper axon morphology in zebrafish.Scientific reports · 2016Article
- α-COP binding to the survival motor neuron protein SMN is required for neuronal process outgrowth.Human molecular genetics · 2015Article
- Repeated low doses of morpholino antisense oligomer: an intermediate mouse model of spinal muscular atrophy to explore the window of therapeutic response.Human molecular genetics · 2015Article
- Effective heritable gene knockdown in zebrafish using synthetic microRNAs.Nature communications · 2015Article
- Spinal Muscular Atrophy Patient iPSC-Derived Motor Neurons Have Reduced Expression of Proteins Important in Neuronal Development.Frontiers in cellular neuroscience · 2015Article
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
Low levels of the survival motor neuron protein (SMN) cause the disease spinal muscular atrophy. A primary characteristic of this disease is motoneuron dysfunction and paralysis. Understanding why motoneurons are affected by low levels of SMN will lend insight into this disease and to motoneuron biology in general. Motoneurons in zebrafish smn mutants develop abnormally; however, it is unclear where Smn is needed for motoneuron development since it is a ubiquitously expressed protein. We have addressed this issue by expressing human SMN in motoneurons in zebrafish maternal-zygotic (mz) smn mutants. First, we demonstrate that SMN is present in axons, but only during the period of robust motor axon outgrowth. We also conclusively demonstrate that SMN acts cell autonomously in motoneurons for proper motoneuron development. This includes the formation of both axonal and dendritic branches. Analysis of the peripheral nervous system revealed that Schwann cells and dorsal root ganglia (DRG) neurons developed abnormally in mz-smn mutants. Schwann cells did not wrap axons tightly and had expanded nodes of Ranvier. The majority of DRG neurons had abnormally short peripheral axons and later many of them failed to divide and died. Expressing SMN just in motoneurons rescued both of these cell types showing that their failure to develop was secondary to the developmental defects in motoneurons. Driving SMN just in motoneurons did not increase survival of the animal, suggesting that SMN is needed for motoneuron development and motor circuitry, but that SMN in other cells types factors into survival.
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Registered trials
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.