ArticleMuscle & nerve2014
The motor neuron response to SMN1 deficiency in spinal muscular atrophy.
Article in Muscle & nerve, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
20 citing papers in PubMed, 43 citations in OpenAlex.
- Nusinersen in later-onset spinal muscular atrophy: Long-term results from the phase 1/2 studies.Neurology · 2019Trial
- Electrophysiological Changes in Pediatric Spinal Muscular Atrophy: Results From an Observational Study.Muscle & nerve · 2026Observational
- Advancing personalized spinal muscular atrophy care: matching the right biomarker to the right patient at the right time.Journal of neurology · 2025Review
- Intersession Intra-Rater and Inter-Rater Reliability of Myotonometer for Upper and Lower Extremity Muscles in Children with Spinal Muscular Atrophy.Diagnostics (Basel, Switzerland) · 2024Article
- Long-term impact of nusinersen on motor and electrophysiological outcomes in adolescent and adult spinal muscular atrophy: insights from a multicenter retrospective study.Journal of neurology · 2024Article
- Assessing Prevalence and Characteristics of Oro-bulbar Involvement in Children and Adults with SMA Type 2 and 3 Using a Multimodal Approach.Dysphagia · 2023Article
- Differential impact on motor unit characteristics across severities of adult spinal muscular atrophy.Annals of clinical and translational neurology · 2023Article
- Peripheral neuropathies associated with DNA repair disorders.Muscle & nerve · 2023Review
- Identifying Biomarkers of Spinal Muscular Atrophy for Further Development.Journal of neuromuscular diseases · 2023Article
- Motor unit recovery following Smn restoration in mouse models of spinal muscular atrophy.Human molecular genetics · 2022Article
- Spinal muscular atrophy - insights and challenges in the treatment era.Nature reviews. Neurology · 2020Review
- Infants Diagnosed with Spinal Muscular Atrophy and 4 SMN2 Copies through Newborn Screening - Opportunity or Burden?Journal of neuromuscular diseases · 2020Article
- Biomarkers and the Development of a Personalized Medicine Approach in Spinal Muscular Atrophy.Frontiers in neurology · 2019Review
- Article
- The neuromuscular impact of symptomatic SMN restoration in a mouse model of spinal muscular atrophy.Neurobiology of disease · 2016Article
- Positional plasticity in regenerating Amybstoma mexicanum limbs is associated with cell proliferation and pathways of cellular differentiation.BMC developmental biology · 2015Article
- Systemic, postsymptomatic antisense oligonucleotide rescues motor unit maturation delay in a new mouse model for type II/III spinal muscular atrophy.Proceedings of the National Academy of Sciences of the United States of America · 2015Article
- SMN expression is required in motor neurons to rescue electrophysiological deficits in the SMNΔ7 mouse model of SMA.Human molecular genetics · 2015Article
- The Genetics of Spinal Muscular Atrophy: Progress and Challenges.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2015Review
- Spinal muscular atrophy: diagnosis and management in a new therapeutic era.Muscle & nerve · 2015Review
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Authors and funding
11 authors at 7 institutions in 1 country.
Funding
Abstract
introductionThe purpose of this study was to measure and analyze motor unit number estimation (MUNE) values longitudinally in spinal muscular atrophy (SMA).
methodsSixty-two children with SMA types 2 and 3 were observed prospectively for up to 42 months. Longitudinal electrophysiological data were collected, including compound motor action potential (CMAP), single motor unit action potential (SMUP), and MUNE.
resultsSignificant motor neuron loss and compensatory collateral reinnervation were noted at baseline. Over time, there was a significant mean increase in MUNE (4.92 units/year, P = 0.009), a mean decrease in SMUP amplitude (-6.32 μV/year, P = 0.10), and stable CMAP amplitude.
conclusionsThe unexpected longitudinal results differ from findings in amyotrophic lateral sclerosis studies, perhaps indicating that compensatory processes in SMA involve new motor unit development. A better understanding of the mechanisms of motor unit decline and compensation in SMA is important for assessing novel therapeutic strategies and for providing key insights into disease pathophysiology.
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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.