Changes in spinal motor neurons during development
Microarchitectural changes during development of the cerebellar cortex
... ABSTRACT The cerebellum is a highly conserved structure in the Central Nervous System (CNS) of vertebrates, and is involved in the coordination of voluntary motor behaviour. Supporting this function, the ...
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How to make spinal motor neurons
... Concluding remarks and perspectives Decades of research into CNS development and spinal MN specification have been synthesized to allow the in vitro generation of MNs through diverse methods. These cells ...
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Identification of STAM1 as a novel effector of ventral projection of spinal motor neurons
... ABSTRACT During spinal cord development, motor neuron (MN) axons exit the spinal cord ventrally, although the molecular basis for this process remains poorly ...in spinal MNs is ...
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Microhexagon gradient array directs spatial diversification of spinal motor neurons
... induce spinal motor neuron diversification and organization, the technology could be further applied as a developmental platform as well as to improve disease modeling of acute spinal cord injury ...
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Spinal muscular atrophy patient iPSC derived motor neurons have reduced expression of proteins important in neuronal development
... and Motor Neuron Differentiation The SMA patient and control subject iPSCs were grown until ∼90% confluent as colonies under normal maintenance conditions before the start of the ...lower spinal ...
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Glycoconjugates Distribution during Developing Mouse Spinal Cord Motor Organizers
... Lectin histochemistry is one of the best experimental procedures for determining the distribution of glycoconjugates and their changes on the cell surface during embryonic development. The ...
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Transcriptome profiling of spinal muscular atrophy motor neurons derived from mouse embryonic stem cells.
... In conclusion, we have identified distinct gene expression patterns in SMA MNs when compared to normal MNs. Pathways upregulated in SMA mESC-derived MNs were involved in pluripotency and cell proliferation whereas common ...
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Microglia and motor neurons during disease progression in the SOD1G93A mouse model of amyotrophic lateral sclerosis: changes in arginase1 and inducible nitric oxide synthase
... the development of overt stride par- ameter differences at 18 weeks of age in the current study correlates well with the transition into a rapidly progressing phase of disease at approximately 18 weeks of age, as ...
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Lmx1b is required for the glutamatergic fates of a subset of spinal cord neurons
... of spinal glutamatergic cells at later stages of ...of spinal glutamatergic cells in the spinal cord in lmx1bb mutants, but by 36 h there is a reduction in the number of glutamatergic cells and this ...
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Pathological changes of distal motor neurons after complete spinal cord injury
... Traumatic spinal cord injury (SCI) causes serious disruption of neuronal circuits that leads to motor functional ...caudal spinal cord has not been sufficiently ...biological changes in the ...
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Aberrant microRNA Expression in Spinal Muscular Atrophy Motor Neurons
... significant genes in each murine model – 5 for Fmr1-KO and 9 for Tsc2+/−. Although 1,486 probe sets were signifi- cantly differentially expressed between WT mice of two different backgrounds, none of these genes was a ...
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Is spinal muscular atrophy a disease of the motor neurons only: pathogenesis and therapeutic implications?
... The most common metabolic defect reported consists of alterations in fatty acid metabolism, which occurs in severe patients and some younger SMA Type II patients [143,144]. Abnormal fatty acid metabolism is characterized ...
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Genetic ablation of dynactin p150Glued in postnatal neurons causes preferential degeneration of spinal motor neurons in aged mice
... Abnormal accumulation of acetylated α -tubulin, and autophagosome/lysosome proteins was found in the SMNs of aged cKO mice. Additionally, the total and cell surface levels of glutamate receptors were also substantially ...
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Transgenic approaches to studying the development of sensory and spinal cord neurons
... of neurons eliminated in a particular DRG, in the absence of each neurotrophin or functional Trk receptor, far exceeded the total number of surviving neurons in that particular wildtype ...sensory ...
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Degeneration of spinal motor neurons by chronic AMPA-induced excitotoxicity in vivo and protection by energy substrates
... of spinal MNs in the rat in vivo and that this results in progressive ...lumbar spinal cord, by means of microdialysis [14,15] or osmotic minipumps [16], respectively, results in MN degeneration that ...
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Motor Neuron-Specific Restoration of SMN in Two SMA Mouse Models: Insights into the Role of Motor Neurons in Spinal Muscular Atrophy
... and motor behavior of SMA 7 mice [78, ...non-motor neurons in severe SMA models, such as reduction of synaptic bouton area, sensory projection dysfunctions, central synaptic defects, affected ...
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Descending Systems Direct Development of Key Spinal Motor Circuits
... mature spinal motor circuits is dependent on both activity-dependent and independent mechanisms during postnatal ...development. During this time, reorganization and refinement of ...
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Dynamic changes of CX3CL1/CX3CR1 axis during microglial activation and motor neuron loss in the spinal cord of ALS mouse model
... dynamic changes of CX3CL1/CX3CR1 axis might be associated with alteration of microglial phenotype as ALS progression in the TG ...manner. During the early disease stage CX3CR1 may be a protective ...
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AMPA Receptor Phosphorylation and Synaptic Colocalization on Motor Neurons Drive Maladaptive Plasticity below Complete Spinal Cord Injury.
... injured spinal cord, while reducing synaptic GluA2. These changes predict motor dysfunction in the absence of cell death signaling, suggesting an opportunity for therapeutic ...horn motor ...
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Cellular bases of the RNA metabolism dysfunction in motor neurons of a murine model of spinal muscular atrophy: Role of Cajal bodies and the nucleolus
... PARGs. During the symptomatic stage, this response is associated with an increased proportion of intron-containing Chat, Chodl, Myh9 and Myh14 mRNAs, suggesting that the formation of PARGs correlates with a ...
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