Search results for "Motoneurons"
showing 5 items of 5 documents
The serum level of free testosterone is reduced in amyotrophic lateral sclerosis
2002
Sporadic amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder affecting upper and lower motoneurons. There is an approximately 2:1 higher incidence of ALS in men compared to women, and this has raised the hypothesis of an involvement of sex hormones in the etiopathogenesis of the disorder. In this work, the serum levels of dehydroepiandrosterone sulphate (DHEAS), 17-betaestradiol, free and total testosterone were measured in 35 patients with defined or probable ALS, according to the El-Escorial/WFN revisited criteria, and compared to those obtained from 57 disease controls, matched for age and gender to the ALS group. We found no differences between ALS cases and …
The Drosophila Hox gene Ultrabithorax acts both in muscles and motoneurons to orchestrate formation of specific neuromuscular connections
2016
Hox genes are known to specify motoneuron pools in the developing vertebrate spinal cord and to control motoneuronal targeting in several species. However, the mechanisms controlling axial diversification of muscle innervation patterns are still largely unknown. We present data showing that the Drosophila Hox gene Ultrabithorax (Ubx) acts in the late embryo to establish target specificity of ventrally projecting RP motoneurons. In abdominal segments A2 to A7, RP motoneurons innervate the ventrolateral muscles VL1-4, with VL1 and VL2 being innervated in a Wnt4-dependent manner. In Ubx mutants, these motoneurons fail to make correct contacts with muscle VL1, a phenotype partially resembling t…
Grp78 overexpression triggers pink1-ip3 r-mediated neuroprotective mitophagy
2021
An experimental model of spinal root avulsion (RA) is useful to study causal molecular programs that drive retrograde neurodegeneration after neuron-target disconnection. This neurode-generative process shares common characteristics with neuronal disease-related processes such as the presence of endoplasmic reticulum (ER) stress and autophagy flux blockage. We previously found that the overexpression of GRP78 promoted motoneuronal neuroprotection after RA. After that, we aimed to unravel the underlying mechanism by carrying out a comparative unbiased proteomic analysis and pharmacological and genetic interventions. Unexpectedly, mitochondrial factors turned out to be most altered when GRP78…
The Etiology of Muscle Fatigue Differs between Two Electrical Stimulation Protocols
2016
International audience; Purpose: This study aimed at investigating the mechanisms involved in the force reduction induced by two electrical stimulation (ES) protocols that were designed to activate motor units differently. Methods: The triceps surae of 11 healthy subjects (8 men; age, similar to 28 yr) was activated using ES applied over the tibial nerve. Two ES protocols (conventional [CONV]: 20 Hz, 0.05 ms vs wide-pulse high-frequency [WPHF]: 80 Hz, 1 ms) were performed and involved 40 trains (6 s on-6 s off) delivered at an intensity (I-ES) evoking 20% of maximal voluntary contraction. To analyze the mechanical properties of the motor units activated at I-ES, force-frequency relation was…
Architecture of cell differentiation, stress-mediated protein expression and transport in iPSC-derived Motor Neurons bearing a pG376D TDP-43 mutation
2020
BACKGROUND AND RATIONALE. ALS is a fatal neurodegenerative disease caused by loss of motoneurons (MNs). Growingevidence suggests that the disease has a strong genetic basis. Up to now, more than 50 ALS-related genes have beenfound. Among these, TARDBP plays a critical role in ALS. Pathogenic mutations in TARDBP gene are found in 1–3% offamilial ALS cases. A few years ago, a novel G376D TARDBP mutation associated to ALS was reported (Conforti et al, 2011). Recently, a verylarge genealogic tree from this G376D family was made (2HE Association, personal communication) leading to a largecollection of DNA, plasma and fibroblasts (FBs) from several family members, both affected and not clinically…