ライフサイエンスコーパス: neuron degeneration

1 sis (ALS) is a fatal disease involving motor neuron degeneration.

2 omplex, NEMF/Rqc2, develop progressive motor neuron degeneration.

3 ned if 6-hydroxydopamine SN lesions cause PF neuron degeneration.

4 /VCP and genetically distinct forms of motor neuron degeneration.

5 r of alpha-syn accumulation and dopaminergic neuron degeneration.

6 t a necessary step in the emergence of motor neuron degeneration.

7 and may contribute to midbrain dopamine (DA) neuron degeneration.

8 ction mechanisms, leading to selective motor neuron degeneration.

9 target for therapeutic manipulation in motor neuron degeneration.

10 ir results may help uncover a cause of motor neuron degeneration.

11 damage response, innate immune response and neuron degeneration.

12 ha-syn in vitro, and caused primary cortical neuron degeneration.

13 ination/protein degradation pathway in motor neuron degeneration.

14 sclerosis (ALS) is a fatal disorder of motor neuron degeneration.

15 uscular junctions, muscle atrophy, and motor neuron degeneration.

16 optotic pathway, would protect against motor neuron degeneration.

17 ALS and an early disease indicator of motor neuron degeneration.

18 roinflammation as an initiation factor of DA neuron degeneration.

19 ular homeostasis that ultimately cause motor neuron degeneration.

20 neurological disorder characterized by motor neuron degeneration.

21 mic inclusions and predominantly lower motor neuron degeneration.

22 the contiguously progressive nature of motor neuron degeneration.

23 , we show exacerbation of selective striatal neuron degeneration.

24 s that a common mechanism may underlie motor neuron degeneration.

25 be manipulated genetically to suppress motor neuron degeneration.

26 ce, the mouse model of SMARD1, lead to motor neuron degeneration.

27 tical and striatal atrophy and striatal dark neuron degeneration.

28 a central contributor to initiation of motor neuron degeneration.

29 2O2, agents that are known to be involved in neuron degeneration.

30 and are a potential target to prevent motor neuron degeneration.

31 logy for identifying genes relevant to motor neuron degeneration.

32 spinal motor neurons does not provoke motor neuron degeneration.

33 associated with increased olfactory sensory neuron degeneration.

34 unction of p150(glued) and accelerates motor neuron degeneration.

35 of skeletal muscle as a consequence of motor neuron degeneration.

36 on is a potential mechanism for dopaminergic neuron degeneration.

37 a fatal adult human disease caused by motor neuron degeneration.

38 erative disease characterized by lower motor neuron degeneration.

39 ecular-weight heat shock proteins, and focal neuron degeneration.

40 in sensory feedback before evidence of motor neuron degeneration.

41 d is implicated in the pathogenesis of motor neuron degeneration.

42 scular disorder characterized by lower motor neuron degeneration.

43 e human genome is theorized to lead to motor neuron degeneration.

44 is colocalized with markers of astrocyte and neuron degeneration.

45 t symptoms; none halt or retard dopaminergic neuron degeneration.

46 ibits both adult lethality and photoreceptor neuron degeneration.

47 binding factors in the pathogenesis of motor neuron degeneration.

48 of PEDF as a neuroprotectant in human motor neuron degeneration.

49 r, suggesting a decrease in the extent of DA neuron degeneration.

50 nities for understanding mechanisms of motor neuron degeneration.

51 This increase precedes motor neuron degeneration.

52 l Muscular Atrophy (SMA), a disease of motor neuron degeneration.

53 myloid plaques, neurofibrillary tangles, and neuron degeneration.

54 neurodegenerative disease that causes motor neuron degeneration.

55 -hydroxydopamine mouse model of dopaminergic neuron degeneration.

56 microtubule cytoskeletons, leading to motor neuron degeneration.

57 isease mechanism underlying axonal and motor neuron degeneration.

58 euromuscular junction denervation, and motor neuron degeneration.

59 in the brain and spinal cord, delaying motor neuron degeneration.

60 infant mortality, is characterized by motor neuron degeneration.

61 its that eventually may be determining motor neuron degeneration.

62 tanding of the complex pathogenesis of motor neuron degeneration.

63 of the disease with very subtle dopaminergic neuron degeneration.

64 in the physiopathological continuum of motor neuron degeneration.

65 an spastic paraplegia characterized by motor neuron degeneration.

66 onal excitability were associated with motor neuron degeneration.

67 S inclusions in the cytoplasm triggers motor neuron degeneration.

68 adult-onset muscle weakness and lower motor neuron degeneration.

69 the role of aberrant RNA processing in motor neuron degeneration.

70 , higher numbers of astrocytes, and Purkinje neuron degeneration.

71 a-synuclein and causes midbrain dopaminergic neuron degeneration.

72 triggered chronic ER stress and dopaminergic neuron degeneration.

73 ad to neuronal dysfunction and ultimately to neuron degeneration.

74 ignificant denervation occurs prior to motor neuron degeneration.

75 ed in disease, which contributes to striatal neuron degeneration.

76 S, disrupt RNA processing and initiate motor neuron degeneration.

77 urodegenerative disease resulting from motor neuron degeneration.

78 cated pathological immune responses in motor neuron degeneration.

79 pinal tract degeneration without lower motor neuron degeneration.

80 ing important biochemical processes, such as neuron degeneration.

81 cs, loss of functional motor units and motor neuron degeneration.

82 ubiquitous SMN decrease but selective motor neuron degeneration.

83 t observed in a cell line derived from motor neuron degeneration 2 mice that carry a mutated form of

84 The mouse mutant mnd2 (motor neuron degeneration 2) exhibits muscle wasting, neurodeg

85 al sclerosis (ALS) is characterized by motor neuron degeneration accompanied by aberrant accumulation

86 the affected males suffer progressive motor neuron degeneration accompanied by signs of androgen ins

87 E is efficacious for thwarting glutamatergic neuron degeneration, alleviating interneuron loss and ab

88 Loss of AnkR causes Purkinje neuron degeneration, altered cerebellar physiology, and

89 Loss of AnkR causes Purkinje neuron degeneration, altered cerebellar physiology, and

90 cases showed combined upper and lower motor neuron degeneration (amyotrophic lateral sclerosis).

91 animals also developed age-dependent sensory neuron degeneration, an accumulation of NF subunits in c

92 136 to alanine substitution rescues dopamine neuron degeneration and age-related locomotor deficits i

93 ses reduced motor function, NMJ alterations, neuron degeneration and altered in vitro stress granule

94 human primates (NHPs), including evidence of neuron degeneration and apoptosis.

95 An autopsy study of one patient showed motor neuron degeneration and axonal loss in the ventral horn

96 osis (ALS); a disease characterized by motor neuron degeneration and cell death.

97 astrocytes is an important driver for motor neuron degeneration and clinical phenotypes of ALS.

98 emphasizes the importance of ATXN2 in motor neuron degeneration and confirms ATXN2 as a therapeutic

99 neurons and/or muscles contributes to motor neuron degeneration and death is poorly understood.

100 lete phosphorylated/activated Akt and permit neuron degeneration and death.

101 mB, and to a lesser extent DeltaDomA, caused neuron degeneration and demyelination in mice infected i

102 Age of onset, pattern of motor neuron degeneration and disease progression vary widely

103 us diseases characterized primarily by motor neuron degeneration and distal weakness.

104 complete loss of eEF1A2 in mice causes motor neuron degeneration and early death; on the other hand h

105 enes implicated in the pathogenesis of motor neuron degeneration and how this new information is chan

106 l discharges always produced extensive hilar neuron degeneration and immediate granule cell disinhibi

107 oteasome system (UPS) has been implicated in neuron degeneration and in pathogenesis of PD.

108 d by decreased strength and endurance, motor neuron degeneration and loss, and denervation of muscle.

109 Motor neuron degeneration and malnutrition alter body composit

110 disease phenotype, with spontaneous striatal neuron degeneration and motor deficits, as assessed by r

111 chanisms of VPS35-D620N-induced dopaminergic neuron degeneration and motor dysfunction via disruption

112 in synaptic clefts, and led to dopaminergic neuron degeneration and motor dysfunction.

113 he SMARD1 disease phenotype, including motor neuron degeneration and muscle atrophy.

114 (SMN) protein and is characterized by motor neuron degeneration and muscle atrophy.

115 erative disorder marked by progressive motor neuron degeneration and muscle denervation.

116 bly to fatal paralysis associated with motor neuron degeneration and muscular atrophy.

117 G toxicity after AAV delivery, including DRG neuron degeneration and necrosis and nerve-fiber degener

118 ing performance may be attributed to reduced neuron degeneration and neuroinflammation.

119 Taken together, the dramatic motor neuron degeneration and paralysis induced by Gpx4 ablati

120 ich increased mtDNA deletions may lead to DA neuron degeneration and parkinsonism.

121 tosomal recessive disease resulting in motor neuron degeneration and progressive life-limiting motor

122 neurological disorder characterized by motor neuron degeneration and progressive muscle paralysis.

123 s extends the VCP phenotype to include motor neuron degeneration and provides another molecular tool

124 ere correlated with ameliorated spinal motor neuron degeneration and reduced lipid peroxidation.

125 Motor neuron degeneration and skeletal muscle atrophy are hall

126 Mutations in BPAG1 cause sensory neuron degeneration and skin fragility in mice.

127 termini is a key factor in preventing motor neuron degeneration and that Bchs reveals a functional l

128 ed skeletal muscle morphology, and prevented neuron degeneration and the development of motor deficit

129 endothelial damage accumulated before motor neuron degeneration and the neurovascular inflammatory r

130 peripherin appear to play some part in motor neuron degeneration, and amyotrophic lateral sclerosis i

131 te that neuronal VEGF protects against motor neuron degeneration, and may have therapeutic implicatio

132 Parkinson's disease, including dopaminergic neuron degeneration, and provides a useful model to stud

133 extensive pinceau disorganization, Purkinje neuron degeneration, and severe ataxia.

134 del for the screening of drugs against motor neuron degeneration, and that MWT is a powerful tool for

135 n (alsin) may indicate a mechanism for motor-neuron degeneration, as it may include several cell-sign

136 ation causes mitochondrial defects and motor neuron degeneration, as the genetic knockout of p35 in a

137 Studies of experimental motor neuron degeneration attributable to expression of neurof

138 ndem with research studying how dopaminergic neuron degeneration begins, is essential for a full unde

139 used in models of ischaemic injury and motor neuron degeneration, both based in part on glutamate tox

140 exhibiting adult-onset upper and lower motor neuron degeneration, but closer examination revealed ear

141 sed to explain how mutant FUS leads to motor neuron degeneration, but neither has been firmly establi

142 We attempted to attenuate neuron degeneration by blocking the synthesis of the cof

143 microglia and oligodendrocytes promote motor neuron degeneration by inducing inflammation and necropt

144 disorder characterised by progressive motor neuron degeneration) cases are due to mutations in the g

145 neuron-specific isoform JNK3 is required for neuron degeneration caused by SMN deficiency.

146 pathological hallmarks, such as dopaminergic neuron degeneration, cognitive decline, and neuroinflamm

147 (123)I]beta-CIT uptake, a marker of dopamine neuron degeneration, compared with those initially treat

148 thesis that regional differences in Purkinje neuron degeneration could provide novel insights into se

149 reduced body weight but did not induce motor neuron degeneration, defects in motor function, or alter

150 dy inclusions and nigrostriatal dopaminergic neuron degeneration define Parkinson's disease neuropath

151 Reexamination of motor neuron degeneration due to alterations of neurofilament

152 abnormalities of phenotypic mice included SN neuron degeneration, extensive neuroinflammation and enh

153 e levels but did not exacerbate dopaminergic neuron degeneration, findings suggesting that basal leve

154 Enteric neuron degeneration has been observed during aging, and

155 eficiency manifests predominantly with motor neuron degeneration; however, a wealth of emerging data

156 y protected against H(2)O(2)-induced retinal neuron degeneration; however, progesterone had no effect

157 ar mechanisms of TAU- and SOD1-induced motor neuron degeneration identified several new SALS-relevant

158 cient to cause major motor deficits or motor neuron degeneration in a mouse model but predisposes neu

159 or mechanisms whereby PS-1 mutations promote neuron degeneration in AD are unknown.

160 pothesized to cause the characteristic motor-neuron degeneration in affected individuals.

161 Putative mechanisms involved in motor neuron degeneration in ALS include oxidative damage, mit

162 at BSCB breakdown contributes to early motor-neuron degeneration in ALS mice and that restoring BSCB

163 nd the molecular pathways that lead to motor neuron degeneration in ALS patients.

164 s, the pathogenic mechanism underlying motor-neuron degeneration in ALS remains largely obscure.

165 naling pathways that may contribute to motor neuron degeneration in ALS, among which are TGF-beta sig

166 astroglial dysfunction contributes to motor neuron degeneration in ALS, the normal function of TDP-4

167 well as the toxic cascades leading to motor neuron degeneration in ALS.

168 chaperone dysfunction plays a role in motor neuron degeneration in ALS.

169 e/inflammatory responses in amplifying motor neuron degeneration in ALS.

170 may contribute to, but does not cause, motor neuron degeneration in ALS.

171 f glutamate transport, and excitotoxic motor neuron degeneration in ALS.

172 death would contribute to spinal cord motor neuron degeneration in ALS.

173 ted and may be an important driver for motor neuron degeneration in ALS.

174 ortant pathogenic mechanism to trigger motor neuron degeneration in ALS.

175 Mechanisms underlying motor neuron degeneration in amyotrophic lateral sclerosis (AL

176 The mechanisms involved in selective motor neuron degeneration in amyotrophic lateral sclerosis rem

177 impair axonal transport and accelerate motor neuron degeneration in amyotrophic lateral sclerosis.

178 esembling apoptosis is responsible for motor-neuron degeneration in amyotrophic lateral sclerosis.

179 to excitotoxicity that participates in motor neuron degeneration in amyotrophic lateral sclerosis.

180 cytes have been shown to contribute to motor neuron degeneration in amyotrophic lateral sclerosis.

181 tochondria macroautophagy impairments and DA neuron degeneration in an in vivo model based on known P

182 of NFL is a common triggering event of motor neuron degeneration in CMT2E and CMT2F disease.

183 disease process resulting in selective motor neuron degeneration in different disease variants has be

184 reduces dopamine levels and causes dopamine neuron degeneration in Drosophila melanogaster.

185 ty can lead to progressive dopaminergic (DA) neuron degeneration in Drosophila.

186 de dismutase (SOD1) cause preferential motor neuron degeneration in familial amyotrophic lateral scle

187 oxide dismutase (SOD1) mutants lead to motor neuron degeneration in familial amyotrophic lateral scle

188 superoxide dismutase (SOD1) gene cause motor neuron degeneration in familial amyotrophic lateral scle

189 f BBB and BSCB was evident in areas of motor neuron degeneration in G93A mice at both early and late

190 nein heavy chain result in progressive motor neuron degeneration in heterozygous mice, and in homozyg

191 0(glued) subunit of dynactin can cause motor neuron degeneration in humans and mice, which resembles

192 dating the patho-physiology underlying motor neuron degeneration in humans.

193 mitochondrial dysfunction, and spinal motor neuron degeneration in inherited ALS.

194 ated MPTP-induced nigrostriatal dopaminergic neuron degeneration in mice, as well as MPTP-induced JNK

195 signs and symptoms of upper and lower motor neuron degeneration in multiple myotomes, without sensor

196 ological processes involved in dopamine (DA) neuron degeneration in Parkinson's disease (PD) are not

197 Oxidative stress contributes to dopaminergic neuron degeneration in Parkinson's disease.

198 ential of an objective biomarker of dopamine neuron degeneration in PD patients.

199 understanding of the mechanism behind motor neuron degeneration in PLS.

200 ts of estrogen on nigrostriatal dopaminergic neuron degeneration in postmenopausal drug-naive Parkins

201 on are associated with and may promote motor neuron degeneration in sALS.

202 f SBMA using mouse models, the role of motor neuron degeneration in SBMA has not been rigorously inve

203 The molecular mechanisms underlying motor neuron degeneration in SMA remain elusive, as global cel

204 ion of Chodl to the pathophysiology of motor neuron degeneration in SMA.

205 iron in the spinal cord leads to early motor-neuron degeneration in SOD1(G93A) mice at least in part

206 es indicated a similar extent of lower motor neuron degeneration in SODMutM maintained on DR or ad li

207 hat the mutant mice develop age-dependent DA neuron degeneration in substantia nigra accompanied by a

208 nal consequences of this pathology including neuron degeneration in the absence of neuronal tau inclu

209 (ALS) causes adult-onset, progressive motor neuron degeneration in the brain and spinal cord, result

210 c and usually fatal disorder caused by motor-neuron degeneration in the brain and spinal cord.

211 neurons in the spinal cord but had no overt neuron degeneration in the cerebral cortex.

212 ttenuated the magnitude of spinal cord motor neuron degeneration in the FALS-transgenic mice.

213 day (E) 6 and E12; whereas, a peak of motor neuron degeneration in the human spinal cord occurs betw

214 e a promising tool for assessing upper motor neuron degeneration in the lateral CST in patients with

215 n could cause synaptic dysfunction and motor neuron degeneration in the mouse model.

216 le in genomic events contributing to delayed neuron degeneration in the SNpR.

217 bitor significantly reduced the dopaminergic neuron degeneration in this interaction model.

218 The enhancement of RNA-mediated motor neuron degeneration in transgenic mice by mutating a maj

219 light neurofilament (NF-L) RNA causes motor neuron degeneration in transgenic mice, leads to accumul

220 hydrogen peroxide (H(2)O(2))-induced retinal neuron degeneration in vitro, and 2) light-induced photo

221 neurons substantially delayed onset of motor neuron degeneration, increasing disease-free life by 50%

222 neuromuscular phenotype accompanied by motor neuron degeneration, indicating striking recapitulation

223 kout (KO) mice protects against dopaminergic neuron degeneration induced by a mutant human alpha-synu

224 PX4 as a ferroptosis inhibitor, spinal motor neuron degeneration induced by Gpx4 ablation exhibited f

225 e the relationship between RNA oxidation and neuron degeneration induced by various insults, includin

226 le electromyographic evidence of lower motor neuron degeneration into diagnostic criteria has undoubt

227 determine whether nigrostriatal dopaminergic neuron degeneration is associated with changes in PPN me

228 results indicate that the mechanism of GABA neuron degeneration is calcium-dependent and requires ac

229 Since motor neuron degeneration is incremental and cumulative over t

230 ic properties relating to selective dopamine neuron degeneration is needed.

231 mechanism by which these mutants cause motor neuron degeneration is not known.

232 onset and progression of ALS and that motor neuron degeneration is partially reversible, at least in

233 A Cdk5 involvement in motor neuron degeneration is supported by analysis of three SO

234 Although motor neuron degeneration is the signature feature of ALS, it

235 by which HFE H63D might increase the risk of neuron degeneration is unclear.

236 ular atrophy (SMA) is characterized by motor neuron degeneration, it is unclear whether and how much

237 cular atrophy (SBMA) is a rare form of motor neuron degeneration linked to a CAG repeat expansion in

238 s affected motor neurons, resulting in motor neuron degeneration, loss of motor functions, and eventu

239 (tg) plus tottering-leaner (tgla), and motor neuron degeneration (mnd) by Northern blot analysis of b

240 nd may be a useful tool for studies of motor neuron degeneration (MND).

241 wn to lead to parkinsonian features in motor neuron degeneration (mnd2) mice.

242 Mild SMA mice exhibit motor neuron degeneration, muscle atrophy, and abnormal EMGs.

243 ies exist that can prevent progressive motor neuron degeneration, muscle denervation, or paralysis in

244 sease characterized by upper and lower motor neuron degeneration, muscle wasting and paralysis.

245 e disease characterized by progressive motor neuron degeneration, muscle wasting, and eventual paraly

246 ever, the importance of ferroptosis in motor neuron degeneration of ALS remains unclear.

247 similar mechanism has been reported in motor neuron degeneration of amyotrophic lateral sclerosis, ab

248 r locus that rescues the phenotype and motor neuron degeneration of nmd mice.

249 is a primary cause in the cascade leading to neuron degeneration or a secondary event to cell death.

250 tion of the motor cortex without lower motor neuron degeneration or involvement of other brainstem tr

251 ult in any overt phenotype or enhanced motor neuron degeneration or loss.

252 resulting founder (F0) mice developed motor neuron degeneration, others displayed phenotypes consist

253 strating a role of SCYL1 in preventing motor neuron degeneration, our findings clearly establish the

254 e disease characterized by progressive motor neuron degeneration, paralysis, and death.

255 trophic lateral sclerosis (ALS) causes motor neuron degeneration, paralysis, and death.

256 ation of RNA pathways at the center of motor neuron degeneration pathogenesis.

257 on in the mouse midbrain causes dopaminergic neuron degeneration, phosphorylated alpha-synuclein elev

258 manifest progressive, mutant-dependent motor neuron degeneration preceded by early, structural and fu

259 Unexpectedly, the acceleration of motor neuron degeneration precedes the accumulation of mutant

260 anisms of apoptosis may participate in motor neuron degeneration produced by mutant copper/zinc super

261 anisms of apoptosis may participate in motor neuron degeneration produced by mutant superoxide dismut

262 uscle-based routes of communication as motor neuron degeneration progresses, and ultimately, they may

263 fatal neurological disease that causes motor neuron degeneration, progressive motor dysfunction, para

264 ut the precise mechanisms of selective motor neuron degeneration remain unresolved.

265 to PD; yet whether mtDNA deletions cause DA neuron degeneration remains unclear.

266 a primary risk factor for PD, its role in DA neuron degeneration remains unknown.

267 ic lateral sclerosis but how it drives motor neuron degeneration remains unresolved.

268 s also consistent with our finding that GABA neuron degeneration requires the mitochondrial fission g

269 ding mechanisms that lead to selective motor neuron degeneration requires visualization and cellular

270 Motor neuron degeneration resulting from the aggregation of th

271 teral sclerosis (ALS) is a progressive motor neuron degeneration resulting in paralysis and death, us

272 We provide evidence that the photoreceptor neuron degeneration seen in flies expressing mutant huma

273 and hindlimb paralysis associated with motor neuron degeneration, severe muscle wasting, and prematur

274 ophic lateral sclerosis (ALS) involves motor neuron degeneration, skeletal muscle atrophy, paralysis,

275 NPC1-YFP produced in neurons prevented neuron degeneration, slowed reactive glial activity, and

276 xonal transport is sufficient to cause motor neuron degeneration such as that observed in amyotrophic

277 den the phenotype of IBMPFD to include motor neuron degeneration, suggest that VCP mutations may acco

278 genic link between ALS2-deficiency and motor neuron degeneration, suggesting a protective role of als

279 ents with focal muscle weakness due to motor neuron degeneration that becomes generalized, leading to

280 ar years earlier than the overt dopaminergic neuron degeneration that drives motor abnormalities and

281 ed protein, causes a unique pattern of motor neuron degeneration that is associated with the accumula

282 cede, and may contribute to, the later motor neuron degeneration that is characteristic of ALS.

283 een independently linked to the dopaminergic neuron degeneration that underlies Parkinson's disease (

284 FUS, a protein critically involved in motor neuron degeneration) that interacted with LRSAM1.

285 2O2 rapidly induces DNA-SSB and causes motor neuron degeneration, the occurrence of which is dose and

286 acterized by selective upper and lower motor neuron degeneration, the pathogenesis of which is unknow

287 6-OHDA) is often used in animal models of DA neuron degeneration, there have been relatively few stud

288 and dysfunction of a single U2 snRNA causes neuron degeneration through distortion of pre-mRNA splic

289 Motor neuron degeneration was detected in nine patients, inclu

290 euronal connectivity that occur during motor neuron degeneration, we characterized the function and s

291 in/dynactin complex and contributes to motor neuron degeneration, we generated p150(glued) G59S knock

292 ore the link between bioenergetics and motor neuron degeneration, we used a computational model in wh

293 ate-onset neuromuscular phenotype with motor neuron degeneration, were studied.

294 at Zfp106 knockout mice develop severe motor neuron degeneration, which can be suppressed by transgen

295 ostatic plasticity induced by ALS-like motor neuron degeneration, which maintains excitatory potentia

296 (ALS) is characterized by progressive motor neuron degeneration, which ultimately leads to paralysis

297 of the Ighmbp2 cDNA prevented primary motor neuron degeneration, while restoring the normal axonal m

298 lerosis are primarily characterized by motor neuron degeneration with additional involvement of non-n

299 sclerosis (ALS) is a fatal disease of motor neuron degeneration with typical survival of only 2-5 ye

300 trophic Lateral Sclerosis (ALS) causes motor neuron degeneration, with 97% of cases exhibiting TDP-43