Survival of motor neuron
Plastin 3 promotes motor neuron axonal growth and extends survival in a mouse model of spinal muscular atrophy
... monogenic motor neuron disease carried by approximately 1/40 people, and account- ing for 1/6,000 to 1/10,000 live ...of survival of motor neuron (SMN1) gene, which encodes a 294-aa ...
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Plastin 3 Promotes Motor Neuron Axonal Growth and Extends Survival in a Mouse Model of Spinal Muscular Atrophy
... monogenic motor neuron disease carried by approximately 1/40 people, and account- ing for 1/6,000 to 1/10,000 live ...of survival of motor neuron (SMN1) gene, which encodes a 294-aa ...
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Cholestenoic acids regulate motor neuron survival via liver X receptors
... postmitotic motor neurons (20, 37) and required for multiple aspects of motor neuron development, including motor neuron specification, motor column formation, axonal growth, and ...
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IPLEX Administration Improves Motor Neuron Survival and Ameliorates Motor Functions in a Severe Mouse Model of Spinal Muscular Atrophy
... of survival motor neuron (SMN) protein that induce selective loss of α -motor neurons (MNs) in the spinal cord, resulting in progressive muscle atrophy and consequent respiratory ...of ...
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Decreased function of survival motor neuron protein impairs endocytic pathways.
... Fig. 3. Altered localization of presynaptic endocytic proteins in smn-1 loss of function animals. (A-E) Representative images of fluorescently-tagged, presynaptic proteins expressed in the dorsal nerve cord of cholinergic ...
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Progranulin modulates zebrafish motoneuron development in vivoand rescues truncation defects associated with knockdown of Survival motor neuron 1
... mouse motor neu- rons express PGRN [30] we examined the expression of zfPGRN-A at both mRNA and protein levels to deter- mine whether grna is expressed within zebrafish ...
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Original Article Acupuncture at ST36 improves survival and ameliorates motor neuron defects by the activation of autophagy
... In total, 4 mice in each group were anesthe- tized using inhaled isoflurane, and blood was collected from retro-orbital veins, followed with a perfusion with 4% paraformaldehyde. The spi- nal cord tissues were removed ...
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Interferons and IRF-1 Induce Expression of the Survival Motor Neuron (SMN) Genes
... For the analysis of the promoter of the SMN gene in response to IFNs and IRF-1, the plasmids containing the promoter of SMN or SMNc genes in front of the chloramphenicol transferase (CAT[r] ...
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Effects of selective serotonin reuptake inhibitors on motor neuron survival
... Figure 2 Dose response curve of fluoxetine. A) Micrographs of fluoxetine-treated motor neurons. Control = cells untreated and incubated for 24 hours. 0.5x fluoxetine = cells treated with 7 ng/ml fluoxetine and ...
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Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy
... Spinal muscular atrophy (SMA) is a common neuromuscular disorder in humans. In fact, it is the most frequently inherited cause of infant mortality, being the result of mutations in the survival of motor ...
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Molecular Genetics of Autosomal Recessive Spinal Muscular Atrophy
... (1995) A frame-shift deletion in the survival motor neuron gene in Spanish spinal mus- cular atrophy patients. Roy N, Mahadevan MS, McLean M, et al[r] ...
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Oligodendrocytes contribute to motor neuron death in ALS via SOD1 dependent mechanism
... increased survival when the knock down was driven by PDGFR, a marker of oligodendrocyte ...control. Motor neurons were plated onto fully differentiated oligodendrocytes for all ...MN survival ...
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Genetic screen identifies a requirement for SMN in mRNA localisation within the Drosophila oocyte
... Objective: Spinal muscular atrophy (SMA) results from insufficient levels of the survival motor neuron (SMN) protein. Drosophila is conducive to large-scale genetic-modifier screens which can reveal ...
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Riluzole: real-world evidence supports significant extension of median survival times in patients with amyotrophic lateral sclerosis
... of motor neuron disease and is a fatal, degenerative, multisystem disorder affecting upper and/or lower motor neurons in the motor cortex, brain stem, and spinal ...extend survival in ...
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Identification and outcomes of clinical phenotypes in amyotrophic lateral sclerosis/motor neuron disease: Australian National Motor Neuron Disease observational cohort
... ALS/MND. 11 13 In addition, the use of patient registries and cohort studies to monitor disease-speci fi c patient care and outcomes has developed worldwide over the past decade. Evidence from the Irish Registry indicated ...
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Reduction of ephrin-A5 aggravates disease progression in amyotrophic lateral sclerosis
... affects motor neurons in the brainstem, spinal cord and motor ...the motor neuron phenotype in zebrafish and a rodent model of ...of motor axons towards their target limb ...reduced ...
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Cellular therapy in motor neuron disease: A case report
... that survival duration in preclinical studies increased by a period of ...the survival duration of MND patients who underwent intrathecal transplantation of autologous BMMNCs was higher by ...existing ...
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Requirement of enhanced Survival Motoneuron protein imposed during neuromuscular junction maturation
... on survival, an easily quantifiable and relevant SMA phenotype in murine ...on survival, whereas treating mice at P12 enhanced median survival by approx- imately 4 ...altered survival was not ...
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Of SMN in mice and men: a therapeutic opportunity
... affects motor neurons, resulting in progres- sive muscular atrophy and ...insufficient survival motor neuron (SMN) protein levels as a result of homozygous disruption of the SMN1 ...
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Motor Learning Mechanism on the Neuron Scale
... cortical motor areas can also be the middle junctions of sandglass structures (24), whose efferent end could be the basal ganglia, DCNs or ...other motor neurons however are more sensitive to the changing ...
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