ArticleNeurobiology of disease2011
Characterization of a commonly used mouse model of SMA reveals increased seizure susceptibility and heightened fear response in FVB/N mice.
Article in Neurobiology of disease, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 24 citations in OpenAlex.
- Challenging the more-is-better dogma: A precision-optimized AAV gene therapy for SMA.Molecular therapy : the journal of the American Society of Gene Therapy · 2026Article
- Efficacy and preliminary safety assessment of EXG001-307 AAV gene therapy for spinal muscular atrophy.Molecular therapy. Methods & clinical development · 2025Article
- Motor transmission defects with sex differences in a new mouse model of mild spinal muscular atrophy.EBioMedicine · 2020Article
- Hyperexcitability precedes motoneuron loss in theJournal of neurophysiology · 2019Article
- Comprehensive Modeling of Spinal Muscular Atrophy inFrontiers in molecular neuroscience · 2019Article
- HACE1 is essential for astrocyte mitochondrial function and influences Huntington disease phenotypes in vivo.Human molecular genetics · 2018Article
- An enhanced Q175 knock-in mouse model of Huntington disease with higher mutant huntingtin levels and accelerated disease phenotypes.Human molecular genetics · 2016Article
- mGlu5 positive allosteric modulation normalizes synaptic plasticity defects and motor phenotypes in a mouse model of Rett syndrome.Human molecular genetics · 2016Article
- Absence of UCHL 1 function leads to selective motor neuropathy.Annals of clinical and translational neurology · 2016Article
- Lack of Chronic Histologic Lesions Supportive of Sublethal Spontaneous Seizures in FVB/N Mice.Comparative medicine · 2016Article
- Corticospinal Motor Neurons Are Susceptible to Increased ER Stress and Display Profound Degeneration in the Absence of UCHL1 Function.Cerebral cortex (New York, N.Y. : 1991) · 2015Article
- Requirement of enhanced Survival Motoneuron protein imposed during neuromuscular junction maturation.The Journal of clinical investigation · 2014Article
- Absence of an intron splicing silencer in porcine Smn1 intron 7 confers immunity to the exon skipping mutation in human SMN2.PloS one · 2014Article
- The DcpS inhibitor RG3039 improves survival, function and motor unit pathologies in two SMA mouse models.Human molecular genetics · 2013Article
- Limited phenotypic effects of selectively augmenting the SMN protein in the neurons of a mouse model of severe spinal muscular atrophy.PloS one · 2012Article
- Temporal requirement for high SMN expression in SMA mice.Human molecular genetics · 2011Article
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Authors and funding
6 authors at 3 institutions in 1 country.
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Abstract
The SMN2 transgenic mouse, Tg(SMN2)89Ahmb, has emerged as the most widely used in spinal muscular atrophy (SMA) research. Here we clone the genomic integration site of the transgene and demonstrate it to be in intron 4 of the metabotropic glutamate receptor 7 (mGluR7) gene. We found that the integration of this transgene significantly reduced both mGluR7 mRNA and protein levels (24% and 9%, respectively). To determine if phenotypes associated with mGluR7 knockout mice were present in Tg(SMN2)89Ahmb containing mice, we subjected mice homozygous for the transgene to open field and seizure susceptibility tests. When compared to wild type FVB/N mice, Tg(SMN2)89Ahmb(tg/tg) mice exhibited significantly longer times in finding a safe wall-adjacent square (+54s if Smn(+/+), +90s if Smn(+/-)), as well as a significantly higher frequency of generalized seizure in response to a subthreshold dose of pentylenetrazol (0.11 vs 0.45). These findings aid in explaining the sudden unexpected death that occurs within SMA mouse colonies that contain a homozygous Tg(SMN2)89Ahmb transgene. This should be taken into account in pre-clinical studies that utilize this transgene, especially in therapy-treated SMA mice that have extended survival.
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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.