ライフサイエンスコーパス: motor neuron

1 presynaptic sensory neuron and postsynaptic motor neuron.

2 en when both forms are expressed in the same motor neuron.

3 ied in other vertebrate brains, for example, motor neurons.

4 n of AdoMet blocks SG formation in yeast and motor neurons.

5 , as well as synaptic functions of GABAergic motor neurons.

6 f neurons, including neuropeptide-expressing motor neurons.

7 pathway, that increases Wnt production from motor neurons.

8 cular atrophy due to loss of upper and lower motor neurons.

9 rns of activity among diverse populations of motor neurons.

10 delivery to primary sensory neurons but not motor neurons.

11 sensory neurons and in target and non-target motor neurons.

12 biogenesis intermediates in human and murine motor neurons.

13 body-weight gain and better preservation of motor neurons.

14 ations in epidermis, aminergic, sensory, and motor neurons.

15 ty of BD motor neurons compared with control motor neurons.

16 y different sources of input to spinal alpha motor neurons.

17 s and/or strong common synaptic input to the motor neurons.

18 tion occurs early during development in SBMA motor neurons.

19 aling between sensory neurons and non-target motor neurons.

20 is uniquely transported retrogradely within motor neurons.

21 ase characterized by the progressive loss of motor neurons.

22 ctin-1, is knocked down (KD) specifically in motor neurons.

23 units and a similar input-output gain of the motor neurons.

24 re motor deficits with clear degeneration of motor neurons.

25 tes in induced pluripotent stem cell-derived motor neurons.

26 neuronal-like SH-SY5Y cells and iPSC-derived motor neurons.

27 due to disrupted axonal transport in mutant motor neurons.

28 induced pluripotent stem cell (iPSC)-derived motor neurons.

29 refore to infer sources of synaptic input to motor neurons.

30 e characterized by the preferential death of motor neurons.

31 neurons in amphioxus, including a subset of motor neurons.

32 rons, local neurons, projection neurons, and motor neurons.

33 rly recruitment of different types of spinal motor neurons.

34 ablishing recruitment order among vertebrate motor neurons.

35 isoforms of the protein and is autonomous to motor neurons.

36 not all, pharyngeal neurons also classify as motor neurons.

37 cifying anterior-posterior identity in vagus motor neurons.

38 imeric G-protein EGL-30//G(alphaq) acting in motor neurons.

39 s caused by mutation or deletion of survival motor neuron 1 (SMN1) and retention of SMN2 leading to S

40 ozygous mutation or deletion of the survival motor neuron 1 (SMN1) gene.

41 ular disease caused by mutations in survival motor neuron 1 (SMN1).

42 SMA is caused by the loss of survival motor neuron 1 (SMN1).

43 ormally suppressed by inputs from inhibitory motor neurons, a behavior known as tonic inhibition.

44 stable clock to set the spike timing of wing motor neurons, a specialized capability that evolved fro

45 le maturation, as well as synchronization of motor neuron activity and muscle contraction under optog

46 ics musculoskeletal model directly driven by motor neuron activity is a promising approach in rehabil

47 cal derivations and a computational model of motor neuron activity, force, and the EMG signal.

48 SBMA embryonic motor neurons also displayed dysfunctional mitochondria

49 Because motor neuron and muscle properties are tightly linked, w

50 encodes a transcriptional repressor, during motor neuron and OPC formation.

51 ropathologically relevant sites, such as the motor neuron and the growth cone.

52 ive survival of a subset of limb-innervating motor neurons and abnormal migration of V2a interneurons

53 ated the recipient neurons may be excitatory motor neurons and ascending interneurons.

54 induced pluripotent stem cell (iPSC)-derived motor neurons and astrocytes to model early cell type-sp

55 eurons and fibers but not with ChAT-positive motor neurons and fibers.

56 Interactions between motor neurons and glia contribute to motor neuron loss,

57 cells ultimately lead to the degeneration of motor neurons and loss of motor function is a fundamenta

58 examining reprogramming of fibroblasts into motor neurons and multiple other somatic lineages, we fi

59 that leads to premature death due to loss of motor neurons and muscle atrophy.

60 res of induced pluripotent stem cell-derived motor neurons and myotubes from patients with FUS-ALS re

61 efects due to intrinsic FUS toxicity in both motor neurons and myotubes.

62 progressive degeneration of upper and lower motor neurons and of neocortical areas, respectively.

63 al cord pMN progenitors sequentially produce motor neurons and oligodendrocyte precursor cells (OPCs)

64 mulation and rescues DNA damage in SMA mice, motor neurons and patient cells.

65 slocalization as well as in patient-specific motor neurons and postmortem patient spinal cord.

66 ctor junction in the gut consists of enteric motor neurons and SIP syncytium, including smooth muscle

67 pluripotent stem cells to generate both the motor neurons and skeletal muscle cells used.

68 apse that is the point of connection between motor neurons and skeletal muscle.

69 We examined ultrastructures of motor neurons and synapses by transmission electron micr

70 egulates L-type CaChs (Dmca1D) in Drosophila motor neurons and this, in turn, controls the release of

71 flow of information from sensory neurons to motor neurons, and apply a recently developed network an

72 DP43 transcripts were enriched in vulnerable motor neurons, and we observed a striking accumulation o

73 e target area of reinnervating corticospinal motor neurons; and a late phase during which growth-prom

74 Next, we show that human iPSC-derived motor neurons are more vulnerable to TDP-43 aggregation

75 joint, and find that only a small number of motor neurons are needed to produce proboscis reaching.

76 During rapid contractions, motor neurons are recruited in a short burst and begin t

77 In the central nervous system, lower motor neurons are selectively affected, whereas patholog

78 report transcripts whose abundances in human motor neurons are sensitive to TDP-43 depletion.

79 Recordings in behaving flies confirmed that motor neurons are typically recruited in order from slow

80 euromuscular diseases, such as regression of motor neuron axons, motor neuron death, and muscle degra

81 terminal activity but does negatively impact motor neuron axons.

82 ron (B21) and its postsynaptic follower, the motor neuron (B8).

83 ovide evidence for three groups of ascending motor neurons based on immunoreactivity and association

84 h each muscle affecting only its presynaptic motor neuron branches.

85 We find that PKM overexpression in the motor neuron, but not the sensory neuron, is sufficient

86 neuromuscular junction (NMJ), the output of motor neurons, but its impact on NMJ repair remains unkn

87 ression was up-regulated in Onecut-deficient motor neurons, but strongly downregulated in Onecut-defi

88 The percent coverage of motor neurons by boutons was reduced by 20%; more specif

89 efore antidromic potentials were elicited in motor neurons by electrical stimulation of a peripheral

90 direct conversion of human fibroblasts into motor neurons by first eradicating fibroblast identity a

91 neuroprotective to seeded aggregation within motor neurons by reducing (mislocalized) cytoplasmic TDP

92 ite projection patterning of the cholinergic motor neuron called PDB in C. elegans.

93 nscriptional and proteomic signatures in ALS motor neurons can vary significantly depending on the id

94 tions and correlates with interneuron versus motor neuron cell fate.

95 including motor function, NMJ pathology and motor neuron cell preservation.

96 can mislocalize to the cytoplasm of affected motor neuron cells, often forming cytoplasmic aggregates

97 pharyngeal neurons into distinct inter- and motor neuron classes, we provide evidence that most phar

98 bserve any changes in the excitability of BD motor neurons compared with control motor neurons.

99 with spinal spheroids, while spinal-derived motor neurons connect with muscle.

100 interact with each other to generate proper motor neuron connectivity.

101 regulation was identified selectively in SMA motor neurons, consistent with previous reports where mi

102 ior to understand how genetically-identified motor neurons control flexion of the fruit fly tibia.

103 Large, fast motor neurons control high force, ballistic movements wh

104 force, ballistic movements while small, slow motor neurons control low force, postural movements.

105 physiological motor unit model consisting of motor neurons coupled to skeletal muscles interacting vi

106 Corticospinal motor neurons (CSMN) and callosal projection neurons (CP

107 We found in C. elegans that GABAergic motor neurons (D-MNs) bias toward the reward behavior in

108 inhibition of p53, which is responsible for motor neuron death and 4-AP, results in additive benefic

109 apeutic target to prevent astrocyte-mediated motor neuron death in ALS.

110 pharmacological inhibition of p53-dependent motor neuron death results in additive effects, leading

111 s, such as regression of motor neuron axons, motor neuron death, and muscle degradation and atrophy c

112 ive disease, characterized by synaptic loss, motor neuron death, and reduced neuronal activity in spi

113 xpressed protein SMN and is characterized by motor neuron death, skeletal muscle atrophy, as well as

114 junctions (NMJs), while having no effects on motor neuron death.

115 ALS) is a neurodegenerative disease in which motor neurons degenerate, resulting in muscle atrophy, p

116 t also emphasizes the importance of ATXN2 in motor neuron degeneration and confirms ATXN2 as a therap

117 ogeneous diseases characterized primarily by motor neuron degeneration and distal weakness.

118 s that astroglial dysfunction contributes to motor neuron degeneration in ALS, the normal function of

119 Mechanisms underlying motor neuron degeneration in amyotrophic lateral scleros

120 e the disease process resulting in selective motor neuron degeneration in different disease variants

121 ful model for the screening of drugs against motor neuron degeneration, and that MWT is a powerful to

122 c homeostatic plasticity induced by ALS-like motor neuron degeneration, which maintains excitatory po

123 RQC complex, NEMF/Rqc2, develop progressive motor neuron degeneration.

124 sclerosis (ALS) is a fatal disease involving motor neuron degeneration.

125 nd heterogeneous group of upper and/or lower motor neuron degenerative disorders, in which the partic

126 vivo intracellular recordings from forelimb motor neurons demonstrated increased corticoreticulospin

127 ontains a transcription factor essential for motor neuron development in Drosophila [6].

128 g and regulatory activity during an in vitro motor neuron differentiation system that recapitulates e

129 This paradigm uses the timings of motor neuron discharges decoded by high-density surface

130 d cognitive and/or behavioural impairment in motor neuron disease (MND).

131 ular atrophy (SMA) is an autosomal recessive motor neuron disease caused by deletion or mutation of S

132 he clinical and scientific network of German motor neuron disease centers (ALS/MND-NET).

133 In addition to cerebellar ataxia, motor neuron disease is often seen in SCA2, and ATXN2 CA

134 ion of this structure as a primary driver in motor neuron disease pathogenesis remains uncertain.

135 hic lateral sclerosis (ALS) is a progressive motor neuron disease that culminates in paralysis and de

136 impairment (MNDci)), behavioural impairment (motor neuron disease with behavioural impairment (MNDbi)

137 e with behavioural impairment (MNDbi), both (motor neuron disease with cognitive and behavioural impa

138 entified patients with cognitive impairment (motor neuron disease with cognitive impairment (MNDci)),

139 a, Alzheimer's disease, Parkinson's disease, motor neuron disease, epilepsy, and Guillain-Barre syndr

140 modulation will help slow the progression of motor neuron disease, offering a novel treatment paradig

141 tive and behavioural impairment (MNDcbi)) or motor neuron disease-frontotemporal dementia (MND-FTD).

142 Spinal muscular atrophy (SMA) is a motor neuron disease.

143 mutation in Vps54 (GARP protein) that causes motor neuron disease.

144 Motor neuron diseases (MNDs) encompass an extensive and

145 ntotemporal lobar degeneration are incurable motor neuron diseases associated with muscle weakness, p

146 a, Alzheimer's disease, Parkinson's disease, motor neuron diseases or epilepsy.

147 -binding and strengthen the link between two motor neuron diseases, ALS and spinal muscular atrophy (

148 a, Alzheimer's disease, Parkinson's disease, motor neuron diseases, epilepsy, or Guillain-Barre.

149 potential disease modifier across different motor neuron diseases, including SMA.

150 a, Alzheimer's disease, Parkinson's disease, motor neuron diseases, or epilepsy.

151 otein triggers the oft-fatal infantile-onset motor neuron disorder, spinal muscular atrophy (SMA).

152 n HMN/CMT2 genes may also be responsible for motor neuron disorders or other neuromuscular diseases,

153 antly, patterns of gene expression in spinal motor neurons dissected from C9-ALS or sporadic ALS pati

154 tream of the Onecut proteins and involved in motor neuron diversification have remained unidentified.

155 hich the Tbk1 gene is selectively deleted in motor neurons, do not display a neurodegenerative diseas

156 y identifying the activity of populations of motor neurons during contractions at maximal rate of for

157 nsory neurons and both target and non-target motor neurons during synapse formation in culture.

158 we investigated the ATXN2 relationship with motor neuron dysfunction in vivo by comparing spinal cor

159 ly rescue both survival of Smn null mice and motor neuron electrophysiology demonstrating that the es

160 Functional connectivity between motor neuron endplates and muscle fibers is confirmed wi

161 We find that leg motor neurons exhibit a coordinated gradient of anatomic

162 otor function is also compromised, but their motor neurons exhibit only subtle morphological and elec

163 Here, we use zebrafish motor neurons expressing a photoactivatable Rac1 to co-o

164 apsyn could be a necessary adjustment to the motor neuron firing rates that increase around the time

165 ample, sequential agonist-antagonist-agonist motor neuron firing typically underlies the slowing ofte

166 EV-D68 spreads to, infects, and kills spinal motor neurons following infection by various routes of i

167 To address this hypothesis, we analysed motor neurons from a human ALS induced-pluripotent stem

168 vailable multiOMIC data sets generated using motor neurons from ALS patients and control cohorts.

169 dominantly excluded from TDP-43 pathology in motor neurons from individuals with ALS.

170 for conferring optimal protection to spinal motor neurons from ischemic spinal cord injury (ISCI).

171 In contrast, motor neurons from patients with mutations in SOD1 exhib

172 We show in primary motor neurons from TDP-43M337V mice that genetically-dri

173 odegenerative disorder caused by the loss of motor neurons from the brain and spinal cord.

174 impairment of proteins that are involved in motor neuron function and stability, including acetylcho

175 the emerging role of regulatory microRNAs in motor neuron generation in developing embryos and their

176 ransmission in the inhibition of hypoglossal motor neurons (HMNs) during REM sleep.

177 is the most common degenerative disorder of motor neurons in adults.

178 sults in an increase in the number of spinal motor neurons in larvae.

179 ng specifically of corticospinal tract (CST) motor neurons in mice, to identify their 'regenerative t

180 ional states, governs the differentiation of motor neurons in mouse and human, but the tempo at which

181 Furthermore, genes were enriched in SBMA motor neurons in several key pathways including p53, DNA

182 interneurons, AVA, that control cholinergic motor neurons in the avoidance neural circuit.

183 We describe the anatomy of all the primary motor neurons in the fly proboscis and characterize thei

184 hese astrocytic changes do not cause loss of motor neurons in the spinal cord or denervation at the n

185 s a key contributor to the susceptibility of motor neurons in this disorder.

186 netically evoked de- or hyperpolarization of motor neurons increased or eliminated action potential a

187 apes receive direct auditory input and drive motor neurons, input and output pathways for delayed esc

188 3 aggregates can more readily propagate from motor neurons into astrocytes in co-culture paradigms.

189 ption factors control the diversification of motor neurons into distinct neuronal subsets by ensuring

190 chnologies sensitive to both upper and lower motor neuron involvement may ultimately resolve controve

191 n proliferative cells and human iPSC-derived motor neurons (iPS-MNs).

192 (iPSC)-induced excitatory neurons and lower motor neurons, iPSC-derived hippocampal dentate gyrus-li

193 th the C9orf72 HRE expansion in iPSC-derived motor neurons (iPSMNs).

194 ease, characterized by the selective loss of motor neurons leading to paralysis.

195 cular disease characterized by loss of alpha-motor neurons, leading to profound skeletal muscle atrop

196 ociated with spasticity in humans with upper motor neuron lesions.

197 like EEL-1, OGT-1 is expressed in GABAergic motor neurons, localizes to GABAergic presynaptic termin

198 e and motor deficits as well as reduction of motor neuron loss and gliosis.

199 The topography of virus-induced motor neuron loss correlates with the pattern of paralys

200 enerative disease characterized by extensive motor neuron loss leading to paralysis and premature dea

201 a neurodegenerative disease characterized by motor neuron loss that ultimately leads to fatal paralys

202 between motor neurons and glia contribute to motor neuron loss, but the spatiotemporal ordering of mo

203 ncurable neurodegenerative disease caused by motor neuron loss, resulting in muscle wasting, paralysi

204 uromuscular endplates that denervated before motor neuron loss, which is consistent with 'dying-back'

205 pathology, including muscle denervation and motor neuron loss.

206 s, which in turn regulates expression of the motor neuron master transcription factor HB9.

207 urvived, differentiated, acquired markers of motor neuron maturation, and extended betaIII-tubulin-po

208 demonstrates at single-neuron resolution how motor neurons may help shape threat-reward choice behavi

209 The precise pattern of motor neuron (MN) activation is essential for the execut

210 pression levels of both Krox20 and MBP in SC-motor neuron (MN) coculture, which was notably prevented

211 fatal neuromuscular disease characterized by motor neuron (MN) death.

212 s (ALS), immune cells and glia contribute to motor neuron (MN) degeneration.

213 eurological disease with progressive loss of motor neuron (MN) function in the brain and spinal cord.

214 k of spinal muscular atrophy (SMA) is severe motor neuron (MN) loss, which results in muscle weakness

215 eroxide dismutase-1 (mutSOD1) kill wild-type motor neurons (MNs) by an unknown mechanism.

216 molecular mechanism that enables cholinergic motor neurons (MNs) in the C. elegans ventral nerve cord

217 The Hoxc8 gene is expressed by pSNs and motor neurons (MNs) targeting distal forelimb muscles, a

218 terminal selector of C. elegans cholinergic motor neurons (MNs), acts indirectly to prevent alternat

219 Motor neurons (MNs), key components of these circuits, p

220 descending projections and denervates lumbar motor neurons (MNs).

221 n human PSC-derived and mouse primary spinal motor neuron models from a physiological perspective.

222 ts, we provide detailed anatomy of proboscis motor neurons, muscles, and joints.

223 o examine the projections of circular muscle motor neurons, myenteric interneurons, and putative sens

224 TRPM8 is necessary for the increase in motor neuron number of animals raised in cold temperatur

225 ctionally related neurons, including cranial motor neurons of the brainstem, are frequently organised

226 However, loss of TBK1 function in motor neurons of the SOD1(G93A) mouse model of ALS impai

227 by a particular vulnerability of the ventral motor neurons of the spinal cord to decreased SMN.

228 ese results suggest that correlated input to motor neurons of two hand muscles can occur even during

229 These data suggest that Prdm8 regulates the motor neuron-OPC switch by controlling the level of Shh

230 an defects caused by TDP-43 proteinopathy in motor neurons or glia, but not muscle, suggesting that m

231 ation of miR-34, Nrx-IV or Hts-M function in motor neurons or muscle supports a model where presynapt

232 es abnormal organismal morphology, inhibited motor neuron outgrowth, decreased mobility, and shorter

233 spinal cord form selective connections with motor neuron partners, but the strategies that confer su

234 riod of disease development with progressive motor neuron pathology, evident as early as embryonic an

235 activation of Onecut genes in the developing motor neurons, performed RNA-sequencing to identify fact

236 C3-C5 spinal cord is the location of phrenic motor neurons (PhMNs) that are responsible for diaphragm

237 ibutable to changes in synaptic input to the motor neuron pool or to adaptations in intrinsic motor n

238 rrently activated, synaptic input to the two motor neuron pools is shared across all frequency bandwi

239 The observed connectivity indicates that motor neuron pools receive common input even when digit

240 dherins regulate distinct aspects of cranial motor neuron positioning and establish subnuclear topogr

241 ASO designed to correct splicing of survival motor neuron pre-mRNA in the cortex and striatum after i

242 Differentiation of PLS from upper motor neuron-predominant forms of amyotrophic lateral sc

243 stembryonic thoracic lineages, excluding the motor neuron producing lineages (15 and 24) which have b

244 r neuron pool or to adaptations in intrinsic motor neuron properties.

245 R-23a expression in SMA patient iPSC-derived motor neurons protected against degeneration, suggesting

246 also find that fast, intermediate, and slow motor neurons receive distinct proprioceptive feedback s

247 that recurrent interactions are dominated by motor neurons recruited during stronger movements and se

248 iple is not the only mechanism that dictates motor neuron recruitment.

249 on guidance in actively navigating zebrafish motor neurons, regulating calcium signaling and filopodi

250 st that the muscle stem cell plays a role in motor neuron reinnervation, myonuclear accretion, and mi

251 chanism(s) by which EV-D68 spreads to target motor neurons remains unclear.

252 eletal muscle, independent of any changes in motor neurons, represent the primary locus of neuromuscu

253 inhibit axonal regeneration of iPSC-derived motor neurons, rescue of stathmin-2 expression restores

254 evealed that prdm8 mutant embryos have fewer motor neurons resulting from a premature switch from mot

255 rating scale, slow vital capacity, and upper motor neuron score) and between muscle data and clinico-

256 It is unclear why motor neurons selectively degenerate and there are curre

257 In the central nervous system, spinal motor neurons serve as one of the best-characterized cel

258 The overexpression of CIP in the motor neurons significantly improves motor deficits, ext

259 generalized symmetric weakness without upper motor neuron signs.

260 e pathology in SMA mice, including increased motor neuron size, reduced neuromuscular junction pathol

261 the primary cell type that is affected (e.g. motor neurons, skeletal muscle and Schwann cells).

262 circRNAs generated by the Alu-rich Survival Motor Neuron (SMN) genes that code for SMN, an essential

263 lished that deficient expression of survival motor neuron (SMN) protein causes SMA, the molecular pat

264 scleblind was sufficient to restore survival motor neuron (SMN) protein localization in neurons expre

265 etic disorder caused by the loss of survival motor neuron (SMN) protein that leads to premature death

266 Paucity of the survival motor neuron (SMN) protein triggers the oft-fatal infant

267 Here, we demonstrate that survival motor neuron (SMN) protein-the loss of which causes the

268 s caused by deficient expression of survival motor neuron (SMN) protein.

269 iquitous depletion of the essential survival motor neuron (SMN) protein.

270 different members of the family in defining motor neuron spatial organization are not yet clear.

271 C boutons on spinal motor neurons stem from spinal interneurons that express

272 lecular level the positional organization of motor neuron subtypes into discrete nuclear structures t

273 s, including a non-cell autonomous effect on motor neuron survival in ALS.

274 and increased EAAT2 expression, and improved motor neuron survival.

275 Characterised by a slowly progressive upper motor neuron syndrome, the diagnosis is clinical, after

276 entiate neuropeptidergic neurons and promote motor neuron terminal maturation.

277 produces a coordinated output from about 120 motor neurons that control the nematode's muscles.

278 he disease derives solely from dysfunctional motor neurons that may be efficiently targeted by restri

279 very proboscis muscle through control of its motor neurons, the first such collection for an appendag

280 trabithorax (Ubx) in a specific set of adult motor neurons, the NB2-3/lin15 neurons.

281 urons resulting from a premature switch from motor neuron to OPC production.

282 elevated Shh signaling, coincident with the motor neuron to OPC switch.

283 ted mouse embryonic stem cell (mESC) derived motor neurons to explore short and long-term programming

284 tion of Shh signaling restored the number of motor neurons to normal but did not rescue the proportio

285 How pMN progenitors switch from producing motor neurons to OPCs with distinct fates is poorly unde

286 ion of TDP-43 by exposing human iPSC-derived motor neurons to serially passaged sporadic ALS post-mor

287 ver, it is unknown to what extent control of motor neuron-to-muscle synaptic partnerships is a genera

288 control induced astrocytes led to increased motor neuron toxicity in co-cultures, similar to the lev

289 As diverse sensory modalities stimulate motor neurons via acetylcholine, this mechanism enables

290 The activity of spinal motor neurons was assessed by high-density electromyogra

291 ce of innervation by excitatory, cholinergic motor neurons was found.

292 When differentiating this cohort into spinal motor neurons, we did not observe any changes in the exc

293 Here, using primary cortical and motor neurons, we found that excitotoxicity induced the

294 d differentiation of embryonic stem cells to motor neurons, we show that the program runs more than t

295 Fibroblasts and motor neurons were obtained to model the disease and cha

296 dly progressive neurodegenerative disease of motor neurons with a median survival of 2 years.

297 Treatment of ALS-derived motor neurons with AdoMet also suppresses the formation

298 pregulated in flies and patient derived iPSC motor neurons with TDP-43 pathology.

299 rized by the degeneration of upper and lower motor neurons within the brain and spinal cord.

300 trophins may potentially revive degenerating motor neurons, yet this approach is dependent on the pro