Upper motor neurons are a target for gene therapy and UCHL1 is necessary and sufficient to improve cellular integrity of diseased upper motor neurons.
Sign Up to like & getrecommendations! Published in 2021 at "Gene therapy"
DOI: 10.1038/s41434-021-00303-4
Abstract: There are no effective cures for upper motor neuron (UMN) diseases, such as amyotrophic lateral sclerosis (ALS), primary lateral sclerosis, and hereditary spastic paraplegia. Here, we show UMN loss occurs independent of spinal motor neuron… read more here.
Keywords: motor neurons; upper motor; integrity; gene therapy ... See more keywords
Upper motor neuron evaluation in multiple sclerosis patients treated with Sativex®
Sign Up to like & getrecommendations! Published in 2017 at "Acta Neurologica Scandinavica"
DOI: 10.1111/ane.12660
Abstract: Spasticity in multiple sclerosis (MS) results from an imbalance of inputs from descending pathways to the spinal motor circuits, as well as from a damage of the corticospinal tract (CST). read more here.
Keywords: sclerosis; neuron evaluation; motor neuron; upper motor ... See more keywords
Is functional electrical stimulation effective in improving walking in adults with lower limb impairment due to an upper motor neuron lesion? An umbrella review.
Sign Up to like & getrecommendations! Published in 2023 at "Artificial organs"
DOI: 10.1111/aor.14563
Abstract: PURPOSE To conduct an umbrella review of systematic reviews on functional electrical stimulation (FES) to improve walking in adults with an upper motor neuron lesion. METHODS Five electronic databases were searched, focusing on the effect… read more here.
Keywords: electrical stimulation; walking adults; upper motor; functional electrical ... See more keywords
Association of the risk factor UNC13A with survival and upper motor neuron involvement in amyotrophic lateral sclerosis
Sign Up to like & getrecommendations! Published in 2023 at "Frontiers in Aging Neuroscience"
DOI: 10.3389/fnagi.2023.1067954
Abstract: Background The UNC13A gene is an established susceptibility locus for amyotrophic lateral sclerosis (ALS) and a determinant of shorter survival after disease onset, with up to 33.0 months difference in life expectancy for carriers of… read more here.
Keywords: upper motor; motor; neuron involvement; motor neuron ... See more keywords
Unbiased MRI Analyses Identify Micropathologic Differences Between Upper Motor Neuron-Predominant ALS Phenotypes
Sign Up to like & getrecommendations! Published in 2019 at "Frontiers in Neuroscience"
DOI: 10.3389/fnins.2019.00704
Abstract: Amyotrophic lateral sclerosis (ALS) is an incurable and progressively fatal neurodegenerative disease that manifests with distinct clinical phenotypes, which are seen in neuroimaging, and clinical studies. T2- and proton density (PD)-weighted magnetic resonance imaging (MRI)… read more here.
Keywords: upper motor; motor neuron; mri; predominant als ... See more keywords
Mutations and Protein Interaction Landscape Reveal Key Cellular Events Perturbed in Upper Motor Neurons with HSP and PLS
Sign Up to like & getrecommendations! Published in 2021 at "Brain Sciences"
DOI: 10.3390/brainsci11050578
Abstract: Hereditary spastic paraplegia (HSP) and primary lateral sclerosis (PLS) are rare motor neuron diseases, which affect mostly the upper motor neurons (UMNs) in patients. The UMNs display early vulnerability and progressive degeneration, while other cortical… read more here.
Keywords: protein interaction; hsp pls; upper motor; interaction ... See more keywords
Complexity of Generating Mouse Models to Study the Upper Motor Neurons: Let Us Shift Focus from Mice to Neurons
Sign Up to like & getrecommendations! Published in 2019 at "International Journal of Molecular Sciences"
DOI: 10.3390/ijms20163848
Abstract: Motor neuron circuitry is one of the most elaborate circuitries in our body, which ensures voluntary and skilled movement that requires cognitive input. Therefore, both the cortex and the spinal cord are involved. The cortex… read more here.
Keywords: mouse models; motor neurons; complexity generating; upper motor ... See more keywords
Motor cortex analogue neurons in songbirds utilize Kv3 subunits to generate ultranarrow spikes.
Sign Up to like & getrecommendations! Published in 2023 at "eLife"
DOI: 10.7554/elife.81992
Abstract: Complex motor skills in vertebrates require specialized upper motor neurons with precise action potential (AP) firing. To examine how diverse populations of upper motor neurons subserve distinct functions and the specific repertoire of ion channels… read more here.
Keywords: motor neurons; neurons controlling; ultranarrow spikes; upper motor ... See more keywords