Amyotrophic lateral sclerosis (ALS) is the most common adult-onset motor neuron disease. More than 90% of ALS cases are sporadic, and the majority of sporadic ALS patients do not carry mutations in genes causative of familial ALS; therefore, investigation specifically targeting sporadic ALS is needed to discover the pathogenesis. The motor neurons of sporadic ALS patients express unedited GluA2...

Neonatal spinal motor neurons are electrically and dye-coupled by gap junctions, but coupling is transient and disappears rapidly after birth. Here we report that adult motor neurons become recoupled by gap junctions after peripheral nerve injury. One and 4-6 weeks after nerve cut, clusters of dye-coupled motor neurons were observed among axotomized, but not control, lumbar spinal motor neurons...

ZALEWSKI, ANDREW A. Efects of reinnervation on denervated skeletal muscle by axons uf motor, sensory, and sympathtic neurons. Am, J, Physiol. 219(6): 1675-1679. 1970.-Although the effects of denervation on skeletal muscle are reversed after reinnervation by axons of any somatic motor neurons, reinnervation by dendrites of sensory neurons has always proved unsuccessful. In order to determine whe...

Every trunk hemisegment of the zebrafish is innervated by 3 identified primary motoneurons whose development can be observed directly in living embryos. In this paper, we describe another identified neuron that is part of this system. Unlike the other primary motoneurons which are present in all trunk hemisegments, this cell is present in slightly less than half of the trunk hemisegments. Addit...

Loss of the survival motor neuron gene (SMN1) is responsible for spinal muscular atrophy (SMA), the most common inherited cause of infant mortality. Even though the SMA phenotype is traditionally considered as related to spinal motor neuron loss, it remains debated whether the specific targeting of motor neurons could represent the best therapeutic option for the disease. We here investigated, ...

are derived from the ventral dermomyotome. The MMC and LMC can be further subdivided into medial and lateral populations. In the case of the LMC, motor neu-motor neurons project to ventrally derived limb muscles These are exciting times for developmental neurobiolo-Finally, distinct pools of motor neurons are distributed gists. For the first time, a molecular understanding of within these colum...

Controlling motor actions requires online adjustments of time-varying parameters. Although numerous studies have attempted to identify the parameters coded in different motor sites, the relationships between the temporal profile of neuronal responses and the dynamics of motor behavior remain poorly understood in particular because motor parameters such as force and movement direction often chan...

Neuroinflammation is a pathological hallmark of neurodegenerative diseases including amyotrophic lateral sclerosis (ALS), and is characterized by activated microglia at sites of neuronal injury. In ALS, neurons do not die alone; neuronal injury is noncell-autonomous and depends upon a well-orchestrated dialogue between motor neurons and microglia. Evidence from transgenic models expressing muta...

Gene therapy can take advantage of the skeletal muscles/motor neurons anatomical relationship to restrict gene expression to the spinal cord ventral horn. Furthermore, recombinant adenoviruses are attractive viral-vectors as they permit spatial and temporal modulation of transgene expression. In the literature, however, several inconsistencies exist with regard to the intramuscular delivery par...

The Charcot-Marie-Tooth disease 2F (CMT2F) and distal hereditary motor neuropathy 2B (dHMN2B) are caused by autosomal dominantly inherited mutations of the heat shock 27 kDa protein 1 (HSPB1) gene and there are no specific therapies available yet. Here, we assessed the potential therapeutic effect of HDAC6 inhibitors on peripheral neuropathy with HSPB1 mutation using in vitro model of motor neu...