Analysis of Feeding Behavior Characteristics in the Cu/Zn Superoxide Dismutase 1 (SOD1) SOD1G93A Mice Model for Amyotrophic Lateral Sclerosis (ALS)

Kitaoka, Yoshihiro; Seki, Shuichi; Kawata, Sou; Nishiura, Akira; Kawamura, K; Hiraoka, Shin-ichiro; Kogo, Mikihiko; Tanaka, Susumu

doi:10.3390/nu15071651

Cited by 4 publications

(4 citation statements)

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“…Abnormal firing of the MesV is also observed in adult SOD1G93A mice [36]. Furthermore, abnormalities in masticatory rhythms correlate with weight loss in adult SOD1G93A mice [36]. These findings suggest a novel reflex circuit-specific sensory issue, potentially linked to muscle twitching, which could aid in early diagnosis and treatment strategies for ALS [61,100].…”

Section: Characteristic Abnormalities In Mesencephalic Trigeminal Neu...mentioning

confidence: 80%

“…These findings suggest that the abnormalities in the MesV of neonatal ALS model mice may be due to the dysfunction of Na channels [35]. Abnormal firing of the MesV is also observed in adult SOD1G93A mice [36]. Furthermore, abnormalities in masticatory rhythms correlate with weight loss in adult SOD1G93A mice [36].…”

Section: Characteristic Abnormalities In Mesencephalic Trigeminal Neu...mentioning

confidence: 81%

“…MesV neurons are proprioceptive sensory neurons that are required for mastication that not only transmit intrinsic sensations from muscle spindles in jaw movements to the centre, but also directly act on trigeminal motor neurons to control masticatory movements [35,[96][97][98]. Treatment strategies for mastication disorders in ALS may focus on protecting the survival and function of the MesV, which controls mastication movements [35,36,[140][141][142]. Seki et al reported excessively inhibited and irregular neuron-firing activity and a significant decrease in the Na current in the MesV in mouse neonates [115].…”

Section: Treatment Strategies For Als Masticatory Abnormalities Throu...mentioning

confidence: 99%

“…Proprioceptive sensory neurons not only transmit information from muscle spindles to the brain but also act on motoneurons and are involved in the control of muscle movement. Abnormalities in proprioceptive sensory neurons, such as the dorsal root ganglion (DRG) [8,[32][33][34] and mesencephalic trigeminal nucleus (MesV) [35,36], were reported early in ALS research and may represent novel targets for ALS treatment.…”

Section: Introductionmentioning

confidence: 99%

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Characteristics of Sensory Neuron Dysfunction in Amyotrophic Lateral Sclerosis (ALS): Potential for ALS Therapy

Seki,

Kitaoka,

Kawata

et al. 2023

Biomedicines

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Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder characterised by the progressive degeneration of motor neurons, resulting in muscle weakness, paralysis, and, ultimately, death. Presently, no effective treatment for ALS has been established. Although motor neuron dysfunction is a hallmark of ALS, emerging evidence suggests that sensory neurons are also involved in the disease. In clinical research, 30% of patients with ALS had sensory symptoms and abnormal sensory nerve conduction studies in the lower extremities. Peroneal nerve biopsies show histological abnormalities in 90% of the patients. Preclinical research has reported several genetic abnormalities in the sensory neurons of animal models of ALS, as well as in motor neurons. Furthermore, the aggregation of misfolded proteins like TAR DNA-binding protein 43 has been reported in sensory neurons. This review aims to provide a comprehensive description of ALS-related sensory neuron dysfunction, focusing on its clinical changes and underlying mechanisms. Sensory neuron abnormalities in ALS are not limited to somatosensory issues; proprioceptive sensory neurons, such as MesV and DRG neurons, have been reported to form networks with motor neurons and may be involved in motor control. Despite receiving limited attention, sensory neuron abnormalities in ALS hold potential for new therapies targeting proprioceptive sensory neurons.

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Section: Characteristic Abnormalities In Mesencephalic Trigeminal Neu...mentioning

confidence: 80%

Section: Characteristic Abnormalities In Mesencephalic Trigeminal Neu...mentioning

confidence: 81%

Section: Treatment Strategies For Als Masticatory Abnormalities Throu...mentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Characteristics of Sensory Neuron Dysfunction in Amyotrophic Lateral Sclerosis (ALS): Potential for ALS Therapy

Seki,

Kitaoka,

Kawata

et al. 2023

Biomedicines

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Stabilization of V1 interneuron-motor neuron connectivity ameliorates motor phenotype in a mouse model of ALS

Mora,

Stuckert,

von Huth Friis

et al. 2024

Nat Commun

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Loss of connectivity between spinal V1 inhibitory interneurons and motor neurons is found early in disease in the SOD1G93A mice. Such changes in premotor inputs can contribute to homeostatic imbalance of motor neurons. Here, we show that the Extended Synaptotagmin 1 (Esyt1) presynaptic organizer is downregulated in V1 interneurons. V1 restricted overexpression of Esyt1 rescues inhibitory synapses, increases motor neuron survival, and ameliorates motor phenotypes. Two gene therapy approaches overexpressing ESYT1 were investigated; one for local intraspinal delivery, and the other for systemic administration using an AAV-PHP.eB vector delivered intravenously. Improvement of motor functions is observed in both approaches, however systemic administration appears to significantly reduce onset of motor impairment in the SOD1G93A mice in absence of side effects. Altogether, we show that stabilization of V1 synapses by ESYT1 overexpression has the potential to improve motor functions in ALS, demonstrating that interneurons can be a target to attenuate ALS symptoms.

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Preservation of masseter muscle until the end stage in the SOD1G93A mouse model for ALS

Kawata,

Seki,

Nishiura

et al. 2024

Sci Rep

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Amyotrophic lateral sclerosis (ALS) progressively impairs motor neurons, leading to muscle weakness and loss of voluntary muscle control. This study compared the effects of SOD1 mutation on masticatory and limb muscles from disease onset to death in ALS model mice. Notably, limb muscles begin to atrophy soon after ALS-like phenotype appear, whereas masticatory muscles maintain their volume and function in later stages. Our analysis showed that, unlike limb muscles, masticatory muscles retain their normal structure and cell makeup throughout most of the disease course. We found an increase in the number of muscle satellite cells (SCs), which are essential for muscle repair, in masticatory muscles. In addition, we observed no reduction in the number of muscle nuclei and no muscle fibre-type switching in masticatory muscles. This indicates that masticatory muscles have a higher resistance to ALS-related damage than limb muscles, likely because of differences in cell composition and repair mechanisms. Understanding why masticatory muscles are less affected by ALS could lead to the development of new treatments. This study highlights the importance of studying different muscle groups in ALS to clarify disease aetiology and mechanisms.

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Analysis of Feeding Behavior Characteristics in the Cu/Zn Superoxide Dismutase 1 (SOD1) SOD1G93A Mice Model for Amyotrophic Lateral Sclerosis (ALS)

Cited by 4 publications

References 55 publications

Characteristics of Sensory Neuron Dysfunction in Amyotrophic Lateral Sclerosis (ALS): Potential for ALS Therapy

Characteristics of Sensory Neuron Dysfunction in Amyotrophic Lateral Sclerosis (ALS): Potential for ALS Therapy

Stabilization of V1 interneuron-motor neuron connectivity ameliorates motor phenotype in a mouse model of ALS

Preservation of masseter muscle until the end stage in the SOD1G93A mouse model for ALS

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