Topic
Motor neuron
About: Motor neuron is a research topic. Over the lifetime, 7759 publications have been published within this topic receiving 392117 citations. The topic is also known as: motoneuron & efferent neuron.
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TL;DR: Targeted disruption of Olig1 and Olig2 genes sheds light on the ontogeny of oligodendroglia and genetic requirements for their development from multipotent CNS progenitors, indicating that oligod endodendrocytes are derived from Olig-specified progenitor that give rise also to neurons.
1,006 citations
TL;DR: The results suggest that the induction of floor plate cells and motor neurons by the notochord in vivo is mediated by exposure of neural plate cells to different concentrations of the amino-terminal product of SHH autoproteolytic cleavage.
959 citations
TL;DR: The results show that dysfunction of dynactin-mediated transport can lead to human motor neuron disease.
Abstract: Impaired axonal transport in motor neurons has been proposed as a mechanism for neuronal degeneration in motor neuron disease. Here we show linkage of a lower motor neuron disease to a region of 4 Mb at chromosome 2p13. Mutation analysis of a gene in this interval that encodes the largest subunit of the axonal transport protein dynactin showed a single base-pair change resulting in an amino-acid substitution that is predicted to distort the folding of dynactin's microtubule-binding domain. Binding assays show decreased binding of the mutant protein to microtubules. Our results show that dysfunction of dynactin-mediated transport can lead to human motor neuron disease.
946 citations
TL;DR: The ambient SHH concentration during the late period determines whether ventralized progenitors differentiate into motor neurons or interneurons, thus defining the pattern of neuronal cell types generated in the neural tube.
923 citations
TL;DR: It is reported that insulin-like growth factor 1 prolongs life and delays disease progression, even when delivered at the time of overt disease symptoms.
Abstract: Amyotrophic lateral sclerosis (ALS) is a progressive, lethal neuromuscular disease that is associated with the degeneration of spinal and brainstem motor neurons, leading to atrophy of limb, axial, and respiratory muscles. The cause of ALS is unknown, and there is no effective therapy. Neurotrophic factors are candidates for therapeutic evaluation in ALS. Although chronic delivery of molecules to the central nervous system has proven difficult, we recently discovered that adeno-associated virus can be retrogradely transported efficiently from muscle to motor neurons of the spinal cord. We report that insulin-like growth factor 1 prolongs life and delays disease progression, even when delivered at the time of overt disease symptoms.
904 citations