“…BACKGROUND: There are no cures or efficacious treatments for severe motor neuron diseases. It is extremely difficult to obtain naïve spinal motor neurons (sMNs) from human tissues for research due to both technical and ethical reasons. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…During the last 20 years at least 23 cases of motor neuron disease have been reported in HIV-1 seropositive patients. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…The mechanism through which mutant HSPB8 leads to a specific motor neuron disease phenotype is currently unknown. To address this question, we compared the effect of mutant HSPB8 in primary neuronal and glial cell cultures. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…The transgenic animals with mutant copper/zinc superoxide dismutase (SOD1) DNA develop paralytic motor neuron disease resembling human amyotrophic lateral sclerosis (ALS) patients and are commonly used as models for ALS. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a fatal progressive motor neuron disease, for which there are still no diagnostic/prognostic test and therapy. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…Strong relationships were identified as follows: FTD with motor neuron disease and FTLD-TDP; SD and FTLD-TDP; PSPS and FTLD-tau; and CBS and FTLD-tau. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…Mutation of which causes ER stress and motor neuron disease, making it important to determine which proteins bind VAP. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…Cerebral palsy (CP) is an upper motor neuron disease that results in a spectrum of movement disorders. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…INTRODUCTION: An expanded hexanucleotide repeat in the C9ORF72 gene has recently been identified as an important cause of frontotemporal dementia and motor neuron disease; however, the phenotypic spectrum of this entity and its pathophysiologic basis have yet to be fully defined. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…In addition, there is also overlap of FTD with motor neuron disease (FTD-MND or FTD-ALS), as well as the parkinsonian syndromes, progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…Alterations to biometal homeostasis are known to play a critical role in pathology in Alzheimer's, Parkinson's, Huntington's and motor neuron diseases. We have previously shown accumulation of the biometals, zinc, copper, manganese and cobalt, in CLN6 Merino and South Hampshire sheep at the age of symptom onset. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…BACKGROUND: As a disease of motor nervous system (motor neuron disease), amyotrophic lateral sclerosis (ALS) has a great impact on several aspects of quality of life (QoL). …”
Enlace del recurso
Enlace del recurso

“…Mutant VAPB did not codistribute with mutant forms of seipin that are associated with an autosomal dominant motor neuron disease, and accumulate in a protective ER derived compartment termed ERPO (ER protective organelle) in neurons. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…As a side effect of our search for side effects, we also list pathological causes of laughter, among them epilepsy (gelastic seizures), cerebral tumours, Angelman’s syndrome, strokes, multiple sclerosis, and amyotrophic lateral sclerosis or motor neuron disease. Conclusions Laughter is not purely beneficial. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…Abnormal biometal homeostasis is a central feature of many neurodegenerative disorders including Alzheimer's disease (AD), Parkinson's disease (PD), and motor neuron disease. Recent studies have shown that metal complexing compounds behaving as ionophores such as clioquinol and PBT2 have robust therapeutic activity in animal models of neurodegenerative disease; however, the mechanism of neuroprotective action remains unclear. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…Exposures to high levels of environmental selenium have been associated with motor neuron disease in both animals and humans and high levels of selenite have been identified in the cerebrospinal fluid of patients with amyotrophic lateral sclerosis (ALS). …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…Progressive supranuclear palsy, corticobasal degeneration, and motor neuron disease may possess clinical and pathological characteristics that overlap with FTD, and it is possible that they may all belong to the same clinicopathological spectrum. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…Amyotrophic lateral sclerosis (ALS) is the most common adult onset motor neuron disease. The etiology and pathogenic mechanisms of the disease remain unknown, and there is no effective treatment. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…Amyotrophic lateral sclerosis (ALS) is a motor neuron disease characterized by progressive distal axonopathy that precedes actual motor neuron death. …”
Enlace del recurso
Enlace del recurso
Enlace del recurso

“…Proximal spinal muscular atrophy (SMA) is an early onset, autosomal recessive motor neuron disease caused by loss of or mutation in SMN1 (survival motor neuron 1). …”
Enlace del recurso
Enlace del recurso
Enlace del recurso