&lt;em&gt;In Vivo&lt;/em&gt; Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration

Pollari, Eveliina; Prior, Robert; Robberecht, Wim; Damme, Philip Van; Bosch, Ludo Van Den

doi:10.3791/57741-v

Cited by 11 publications

(8 citation statements)

References 9 publications

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“…The protocol of (Pollari et al 2018) was followed for electromyography measurements of muscle. An analogue stimulator (Grass Telefactor W. Waarwick, RI USA model 588) and a stimulus isolation unit (SIU) (model PSIU6 (Grass Instrument Co USA)), plus an AC/DC amplifier model 3000 (A-M Systems) were used.…”

Section: Muscle Cmap (Compound Muscle Action Potential) Measurementsmentioning

confidence: 99%

Mutation in the FUS nuclear localisation signal domain causes neurodevelopmental and systemic metabolic alterations

Ali,

Godoy-Corchuelo,

Martins-Bach

et al. 2023

Disease Models &Amp; Mechanisms

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Mutations in the ubiquitously expressed DNA/RNA binding protein FUS cause aggressive juvenile forms of amyotrophic lateral sclerosis (ALS). While most FUS mutation studies have focused on motor neuron degeneration, little is known about wider systemic or developmental effects. We studied pleiotropic phenotypes in a physiological knock-in mouse model carrying the pathogenic FUSDelta14 mutation in homozygosity. RNA sequencing of multiple organs aimed to identify pathways altered by the mutant protein in the systemic transcriptome, including metabolic tissues given the link between ALS-FTD and altered metabolism. Few genes were commonly altered across all tissues, and most genes and pathways affected were generally tissue-specific. Phenotypic assessment of mice revealed systemic metabolic alterations related to the pathway changes identified. MRI brain scans and histological characterisation revealed that homozygous FUSDelta14 brains were smaller and displayed significant morphological alterations including a thinner cortex, reduced neuronal number and increased gliosis, which correlated with early cognitive impairment and fatal seizures. We show that the disease aetiology of FUS mutations can include both neurodevelopmental and systemic alterations.

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Section: Muscle Cmap (Compound Muscle Action Potential) Measurementsmentioning

confidence: 99%

Mutation in the FUS nuclear localisation signal domain causes neurodevelopmental and systemic metabolic alterations

Ali,

Godoy-Corchuelo,

Martins-Bach

et al. 2023

Disease Models &Amp; Mechanisms

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“…Alternatively, for Nae1 icKO mice, the UltraPro S100 EMG/NCS/EP Neurodiagnostic System (Natus) was used using a protocol adapted from ( 63 ). In brief, Nae1 i cKO mice were anesthetized with isoflurane (5% induction, 2% maintenance).…”

Section: Methodsmentioning

confidence: 99%

Neddylation orchestrates the complex transcriptional and posttranscriptional program that drives Schwann cell myelination

Ayuso-García,

Sánchez-Rueda,

Velasco-Avilés

et al. 2024

Sci. Adv.

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Myelination is essential for neuronal function and health. In peripheral nerves, >100 causative mutations have been identified that cause Charcot-Marie-Tooth disease, a disorder that can affect myelin sheaths. Among these, a number of mutations are related to essential targets of the posttranslational modification neddylation, although how these lead to myelin defects is unclear. Here, we demonstrate that inhibiting neddylation leads to a notable absence of peripheral myelin and axonal loss both in developing and regenerating mouse nerves. Our data indicate that neddylation exerts a global influence on the complex transcriptional and posttranscriptional program by simultaneously regulating the expression and function of multiple essential myelination signals, including the master transcription factor EGR2 and the negative regulators c-Jun and Sox2, and inducing global secondary changes in downstream pathways, including the mTOR and YAP/TAZ signaling pathways. This places neddylation as a critical regulator of myelination and delineates the potential pathogenic mechanisms involved in CMT mutations related to neddylation.

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“…The CMAP is intended to measure the sum of action potentials of the concerned muscle fibers, focusing on the response of the innervated muscle [52]. Notably, the experiment by Di Summa et al (2011) [12] assessed nerve regeneration through significant increases in axonal diameter and CMAP using either autograft or fibrin seeded with ASCs on the PNI.…”

Section: Functional Analysismentioning

confidence: 99%

“…This parameter appears to be used due to the ease of its application in studies, often being the only type of functional evaluation performed [23,29]. In the experimental trial conducted by Pollari et al (2018) [56], the method proved effective in comparing nerve regeneration in the transection versus crushing of the peripheral nerve group.…”

Section: Functional Analysismentioning

confidence: 99%

Evaluation of Postoperative Nerve Regeneration in Murine Experimental Models Subjected to Nerve Transection

Araújo,

Mussalem,

Bani

et al. 2024

Preprint

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Objectives: This study aims to explore the use of murine models in peripheral nerve transection research, evaluating and synthesizing key methods for analyzing nerve regeneration to guide future research and clinical interventions. Methodology: A systematic review was conducted in February 2024, adhering to Cochrane and PRISMA 2020 guidelines, using Medline, Scielo, and Lilacs databases. The focus was on experimental studies of nerve regeneration in animal models post-transection. Only experimental clinical trials reporting nerve regeneration outcomes were included; studies lacking a comparator group or functional evaluation methods were excluded. Results: Out of 273 studies initially identified from MEDLINE, 19 were selected for detailed analysis. The average study included 32.5 subjects, with about 10.16 subjects per intervention subgroup. The predominant model was the sciatic nerve injury with a 10mm gap. The most common intervention involved unprocessed adipose-derived stem cells, utilized in 14 articles. Conclusions: The review underscores the significant potential of current methodologies in peripheral nerve regeneration, particularly highlighting the use of murine models and thorough evaluation techniques. These studies significantly contribute to our understanding of nerve regeneration processes and inform directions for future research.

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<em>In Vivo</em> Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration

Cited by 11 publications

References 9 publications

Mutation in the FUS nuclear localisation signal domain causes neurodevelopmental and systemic metabolic alterations

Mutation in the FUS nuclear localisation signal domain causes neurodevelopmental and systemic metabolic alterations

Neddylation orchestrates the complex transcriptional and posttranscriptional program that drives Schwann cell myelination

Evaluation of Postoperative Nerve Regeneration in Murine Experimental Models Subjected to Nerve Transection

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