ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2013
eGFP expression under UCHL1 promoter genetically labels corticospinal motor neurons and a subpopulation of degeneration-resistant spinal motor neurons in an ALS mouse model.
Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 57 papers.
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57 citing papers in PubMed, 76 citations in OpenAlex.
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- Cell Type-Specific Alterations in Excitability and Inhibition of Upper Motor Neurons in AlsinKO Mice, a Model of Juvenile Onset ALS.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2025Article
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- High-density multielectrode arrays bring cellular resolution to neuronal activity and network analyses of corticospinal motor neurons.Scientific reports · 2025Article
- Development and validation a novel FEZF2 based fluorescent reporter for corticospinal motor neurons.Metabolic brain disease · 2024Article
- Small molecules targeting different cellular pathologies for the treatment of amyotrophic lateral sclerosis.Medicinal research reviews · 2023Review
- Astroglial exosome HepaCAM signaling and ApoE antagonization coordinates early postnatal cortical pyramidal neuronal axon growth and dendritic spine formation.Nature communications · 2023Article
- Novel rAAV vector mediated intrathecal HGF delivery has an impact on neuroimmune modulation in the ALS motor cortex with TDP-43 pathology.Gene therapy · 2023Article
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- Modeling mammalian spermatogonial differentiation and meiotic initiation in vitro.Development (Cambridge, England) · 2022Article
- Caudal-Rostral Progression of Alpha Motoneuron Degeneration in the SOD1Antioxidants (Basel, Switzerland) · 2022Article
- Upper motor neurons are a target for gene therapy and UCHL1 is necessary and sufficient to improve cellular integrity of diseased upper motor neurons.Gene therapy · 2022Article
- NU-9 improves health of hSOD1Scientific reports · 2022Article
- Unique molecular features and cellular responses differentiate two populations of motor cortical layer 5b neurons in a preclinical model of ALS.Cell reports · 2022Article
- Mitochondrial dysregulation occurs early in ALS motor cortex with TDP-43 pathology and suggests maintaining NADScientific reports · 2022Article
- Mapping Motor Neuron Vulnerability in the Neuraxis of Male SOD1Frontiers in endocrinology · 2022Article
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Authors and funding
9 authors at 4 institutions in 2 countries.
Funding
Abstract
Understanding mechanisms that lead to selective motor neuron degeneration requires visualization and cellular identification of vulnerable neurons. Here we report generation and characterization of UCHL1-eGFP and hSOD1(G93A)-UeGFP mice, novel reporter lines for cortical and spinal motor neurons. Corticospinal motor neurons (CSMN) and a subset of spinal motor neurons (SMN) are genetically labeled in UCHL1-eGFP mice, which express eGFP under the UCHL1 promoter. eGFP expression is stable and continues through P800 in vivo. Retrograde labeling, molecular marker expression, electrophysiological analysis, and cortical circuit mapping confirmed CSMN identity of eGFP(+) neurons in the motor cortex. Anatomy, molecular marker expression, and electrophysiological analysis revealed that the eGFP expression is restricted to a subset of small-size SMN that are slow-twitch α and γ motor neurons. Crossbreeding of UCHL1-eGFP and hSOD1(G93A) lines generated hSOD1(G93A)-UeGFP mice, which displayed the disease phenotype observed in a hSOD1(G93A) mouse model of ALS. eGFP(+) SMN showed resistance to degeneration in hSOD1(G93A)-UeGFP mice, and their slow-twitch α and γ motor neuron identity was confirmed. In contrast, eGFP(+) neurons in the motor cortex of hSOD1(G93A)-UeGFP mice recapitulated previously reported progressive CSMN loss and apical dendrite degeneration. Our findings using these two novel reporter lines revealed accumulation of autophagosomes along the apical dendrites of vulnerable CSMN at P60, early symptomatic stage, suggesting autophagy as a potential intrinsic mechanism for CSMN apical dendrite degeneration.
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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.