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6 ly, genetic deletion of Parp1 rescued normal cerebellar ADP-ribose levels and reduced the loss of cer
10 d that the recently discovered basal ganglia-cerebellar anatomical pathway may support the involvemen
11 order diagnosis on different measures of the cerebellar anatomy as well as the effect of possible fac
14 right cerebellar vermis and left lobule V of cerebellar anterior lobe were additionally activated for
22 nd IFN-induced expression in human and mouse cerebellar astrocytes than did cerebral cortical astrocy
25 cohort of hereditary spastic paraplegia and cerebellar ataxia cases (n = 618) for mutations in POLR3
27 and spectrin-associated autosomal recessive cerebellar ataxia Type 1 pathology likely arises from po
28 and spectrin-associated autosomal recessive cerebellar ataxia Type 1, but molecular mechanisms linki
29 ked in two different families to adult-onset cerebellar ataxia, and a de novo truncation mutation res
30 re were defined for each autosomal recessive cerebellar ataxia, and corresponding prediction scores w
31 sing frequency): peripheral neuropathy, 53%; cerebellar ataxia, dysmetria, or dysarthria, 38%; and en
32 ive mutations in WDR81, previously linked to cerebellar ataxia, mental retardation, and disequilibriu
33 ual disability, and many individuals exhibit cerebellar ataxia, subtle facial dysmorphism, strabismus
34 minant parkinsonism and MSA with predominant cerebellar ataxia, which generally correlate with striat
42 s such as hereditary spastic paraplegias and cerebellar ataxias remain genetically unexplained, impli
43 g molecular diagnoses of autosomal recessive cerebellar ataxias, thereby guiding targeted sequencing
45 atter fraction, T2 lesion volume, metrics of cerebellar atrophy and cerebellar lesion volume) and mot
46 sive encephalopathy characterized by extreme cerebellar atrophy due to almost total granule neuron lo
52 netic resonance imaging revealed symmetrical cerebellar atrophy, which appeared progressive, and a pr
54 e, ASO7 localized to Purkinje cells, reduced cerebellar ATXN2 expression below 75% for more than 10 w
56 lls can fire coincident simple spikes during cerebellar behaviours, we varied the proportion (0-20 of
61 grafts of fetal dopaminergic cells, control cerebellar cells, or vehicle bilaterally and were studie
62 t of an ataxic phenotype is linked to severe cerebellar cellular pathology, characterized by nuclear
63 e network and enhanced activity in a cortico-cerebellar circuit correlated with the severity of socia
65 o previous studies, our findings reveal that cerebellar cognitive areas are reached by the largest pr
66 TC-CPC loop provides a substrate for cerebro-cerebellar communication during cognitive processing.
67 ions about the use of corollary discharge in cerebellar computations, we studied it in mice of both s
68 -mortem humans have established that cerebro-cerebellar connections are contralateral to each other a
69 heavy alcohol consumption, whereas increased cerebellar connectivity in HD may have compensatory effe
70 ks is now well established, yet the specific cerebellar contribution to language processing remains u
71 ll layer (GCL) is fundamental to theories of cerebellar contributions to motor control and learning.
74 S: Purkinje cells are the sole output of the cerebellar cortex and fire two distinct types of action
75 e and Golgi cells, is the first stage of the cerebellar cortex and processes spatiotemporal informati
77 eractions among PCs, which suggests that the cerebellar cortex is more functionally diverse than is a
80 s widely available at the input stage of the cerebellar cortex, as required by forward models of cere
81 g with the pruning of climbing fibres in the cerebellar cortex, implicates the climbing fibre collate
90 internal modeling, we hypothesize that right cerebellar Crus I/II supports prediction of upcoming sen
91 between MRI variables of supratentorial and cerebellar damage (grey matter fraction, T2 lesion volum
97 functional magnetic resonance (MR) imaging, cerebellar dentate nuclei (DNs) functional connectivity
99 sensorimotor adaptation of the upper limb, a cerebellar-dependent process restoring movement accuracy
100 n in Purkinje neurons (PNs) is essential for cerebellar development and for motor learning and altere
101 at Wnt5a signaling is a crucial regulator of cerebellar development and would aid in better understan
102 trating that many key mechanisms controlling cerebellar development are likely conserved between mous
104 tern and functional significance of Wnt5a in cerebellar development using Wnt5a(-/-) and Nestin-Cre m
106 erm birth incorporates an increased risk for cerebellar developmental disorders likely contributing t
107 THGr(Ce) below the determined threshold and cerebellar diaschisis in 25 of 26 (96%) cases with THGr(
108 ysed cross-sectional data from 72 cases with cerebellar disease and 36 controls without cerebellar di
110 ent and would aid in better understanding of cerebellar disease pathogenesis caused due to deregulati
113 eed-based method was applied to identify the cerebellar DNs resting-state network; first-level and hi
114 .2 vs 1.7+/-1.7, P<0.001), indicating slower cerebellar dysfunction indexes for FRDA than for SCA.
118 subjects and support the hypotheses that (i) cerebellar efferents target frontal lobe neurons involve
119 t our work using other methodologies showing cerebellar engagement in linguistic prediction and sugge
120 y skill acquisition would be proportional to cerebellar excitability (CBI) changes, whereas later sta
124 l area of the GC of primary neurons from the cerebellar external granule layer of P2 mouse pups of bo
128 background (Atoh1(S193A/lacZ)) exhibit mild cerebellar foliation defects, motor impairments, partial
129 ABSTRACT: Purkinje cells are central to cerebellar function because they form the sole output of
135 for rating cerebellar ataxias: the Composite Cerebellar Functional Severity (CCFS) Scale and Scale fo
136 nstructed a spatially realistic model of the cerebellar GCL and examined how GCL architecture contrib
139 flux and enhanced migration, while in normal cerebellar granule (precursor) cells and MB cells not de
141 rmal granule cells and the transformed human cerebellar granule cell line DAOY, OGR1 promoted express
142 ons that express high levels of Ptchd1 mRNA: cerebellar granule cell precursors and dentate granule c
143 ere we have shown that deletion of Chd7 from cerebellar granule cell progenitors (GCps) results in re
144 ordings and modeling of synaptic activity at cerebellar granule cell to Purkinje cell synapses of mic
146 es to afferent mixing.SIGNIFICANCE STATEMENT Cerebellar granule cells are among the simplest neurons,
148 ave recently found that the proliferation of cerebellar granule neuron precursors is significantly re
149 required for the regulated proliferation of cerebellar granule neuron progenitors (CGNP) and for the
150 sslinking protein as an entry point into the cerebellar granule neuron system in combination with sup
151 ubiquitin-conjugating enzyme UBC13 in rodent cerebellar granule neurons robustly increases the number
152 bited L1-CAM-dependent neurite elongation in cerebellar granule neurons, a pathway previously shown t
154 All VOIs were referenced to a subsection of cerebellar gray matter (cere-crus) as well as a parametr
155 d at 50-60 min after injection, using either cerebellar gray matter (SUVRCB) or whole subcortical whi
157 n the white matter integrity of the pons and cerebellar gray matter volume associated with higher 'p
160 ent meta-analysis aims to assess patterns of cerebellar grey matter atrophy in seven neurodegenerativ
161 hemisphere (THGr(Ce)) and the contralateral cerebellar hemisphere (THGr(Cb))-by its respective contr
162 ontrast, the role of the lateral cerebellum (cerebellar hemispheres and dentate nuclei, DN) is less w
163 e corroborated by significant improvement of cerebellar hypometabolism (statistical parametric mappin
164 We demonstrate that loss of Wnt5a results in cerebellar hypoplasia and depletion of GABAergic and glu
165 contribute to GCp proliferative defects and cerebellar hypoplasia in GCp-specific Chd7 mouse mutants
166 (GCps) results in reduced GCp proliferation, cerebellar hypoplasia, developmental delay, and motor de
171 s with motor cortex (M1) can be estimated as cerebellar inhibition (CBI): a conditioning pulse of tra
174 of pattern separation we built models of the cerebellar input layer with spatially correlated input p
177 dying this non-cortex-mediated basal ganglia-cerebellar interaction could radically change our perspe
178 motor aspects of language, and suggest that cerebellar internal models of linguistic stimuli support
181 t semantic prediction.SIGNIFICANCE STATEMENT Cerebellar involvement in language tasks and language ne
183 on volume, metrics of cerebellar atrophy and cerebellar lesion volume) and motor/cognitive scores.
184 both motor and cognitive performances, with cerebellar lesion volume, cerebellar Lobules VI, Crus I
185 tate lesions without other focal cerebral or cerebellar lesions were detected at term equivalent age
186 sclerosis (MS), testing the contribution of cerebellar lobular atrophy to both motor and cognitive p
187 matter volume in the occipital lobe and left cerebellar lobule VIIb, which is functionally connected
189 iled mapping of clinical dysfunctions to the cerebellar lobules in disease populations is necessary t
190 performances, with cerebellar lesion volume, cerebellar Lobules VI, Crus I and VIIIa atrophy being in
193 ts indicate that learning-related changes in cerebellar-M1 connectivity reflect a somatotopy-specific
197 We explore the associations between altered cerebellar metabolite profiles and brain injury topograp
199 motor phenotype onset, and have progressive cerebellar molecular and neurophysiological (Purkinje ce
200 P2 sumoylation is required for regulation of cerebellar motor function and vocal communication, likel
203 tside the context of learning, modulation of cerebellar-motor cortex connectivity is somatotopically
204 that adaptive learning with the hand affects cerebellar-motor cortex connectivity, not only for the t
206 th alcohol intake suggests a crucial role of cerebellar networks in the generation of symptoms in the
207 ores and the degree to which connectivity in cerebellar networks is "idiosyncratic" in an individual
208 ve processing that requires both frontal and cerebellar networks that are disrupted in patients with
213 ar ADP-ribose levels and reduced the loss of cerebellar neurons and ataxia in Xrcc1-defective mice, i
214 report that numbers of cerebral cortical and cerebellar neurons are decreased and that cerebral corte
217 associated with a deficit in the ability of cerebellar neurons to form synapses and an increased num
221 KEY POINTS: Large premotor neurons of the cerebellar nuclei (CbN cells) integrate synaptic inhibit
222 ABSTRACT: Large projection neurons of the cerebellar nuclei (CbN cells), whose activity generates
224 sion at synapses from Purkinje cells to deep cerebellar nuclei and at vestibular synapses in mice.
225 gically inhibiting the erratic output of the cerebellar nuclei in the mutant mice improved movement.
226 brain stimulation directed to the interposed cerebellar nuclei reduced dystonia-like postures in thes
227 the output structures of the cerebellum, the cerebellar nuclei, integrate their inputs and influence
229 selectively stimulate neurons in the lateral cerebellar nucleus (LCN), a deep cerebellar nucleus that
230 the lateral cerebellar nucleus (LCN), a deep cerebellar nucleus that sends major excitatory output to
231 These findings advance understanding of cerebellar organization in health and sex chromosome ane
232 hods and results clarify the shifts in human cerebellar organization that accompany interwoven variat
233 kdown animals were associated with irregular cerebellar output caused by changes in the intrinsic act
234 nt neurophysiological changes: modulation of cerebellar output mediated in-part by long-term depressi
235 ive properties of subcortical neurons at the cerebellar output stage mediating conditioned behavior.
237 It cannot be excluded, however, that extra-cerebellar pathology contributed to the present findings
238 e calculated for DN-to-pons and DN-to-middle cerebellar peduncle (MCP) ratios in a region-of-interest
239 obus pallidus (GP) in relation to the middle cerebellar peduncle (MCP), pons, and thalamus after repe
240 cleus (n = 10), colliculi (n = 10), superior cerebellar peduncle (n = 7), caudate nucleus (n = 4), wh
241 ior thalamus, substantia nigra, red nucleus, cerebellar peduncle, colliculi, dentate nucleus, and glo
243 ar vermis hypoplasia with elongated superior cerebellar peduncles (mild "molar tooth sign"), typical
248 ions of a biophysically realistic model of a cerebellar Purkinje cell in a pattern recognition task s
250 matic representations through the cerebellum.Cerebellar Purkinje cells (PCs) linearly encode whisker
251 nts, including decreased firing frequency of cerebellar Purkinje cells and a decline in motor functio
252 urons that send climbing fibers to innervate cerebellar Purkinje cells for the control of motor learn
253 he limbic forebrain, the locus coeruleus and cerebellar Purkinje cells, or for CRF2 in any aspect of
257 fined as 11C-Pittsburgh compound B target-to-cerebellar ratio above 1.5 within a composite cortical v
261 ted activations in dorsal frontoparietal and cerebellar regions, and task-related deactivations in de
263 rebrocortical morphology and effect sizes of cerebellar relative to cerebral morphological difference
265 he Montreal Cognitive Assessment) and normal cerebellar, sensory, cranial nerve, and autonomic functi
268 n cultured mouse granule neurons and ex vivo cerebellar slices indicate that ZNHIT3 is indispensable
271 more valid and accurate resolution of which cerebellar subcomponents are sensitive to sex and sex ch
273 role for the AMPA receptor subunit GluA3 in cerebellar synaptic plasticity and motor learning in mic
274 ement, and brain size.SIGNIFICANCE STATEMENT Cerebellar systems are implicated in diverse domains of
276 ntal cortex and left hippocampus, along with cerebellar, temporal and parietal regions were more subs
277 ine connectivity was correlated with recall, cerebellar-thalamic baseline connectivity was correlated
278 s about the involvement of the basal ganglia-cerebellar-thalamo-cortical system in tic generation; (i
279 d TRPC4 are prominently expressed in healthy cerebellar tissue throughout postnatal development and i
283 predominantly parkinsonism [MSA-P], n = 9), cerebellar type MSA (MSA-C, n = 7), PSP (n = 13), and PD
284 other causes being alcohol excess (12%) and cerebellar variant of multiple system atrophy (11%).
289 The children presented congenital ataxia and cerebellar vermis hypoplasia with elongated superior cer
290 f-generated head movements in the rat caudal cerebellar vermis, an area essential for graviceptive fu
291 sm spectrum disorder diagnosis and increased cerebellar volume (p = .049, uncorrected), but the analy
292 gated scaling relationships between regional cerebellar volume and brain size in humans, which (1) ar
293 rther observed positive correlations between cerebellar volume and cerebral cortical thickness in fro
294 sed candidate endophenotypes, differences in cerebellar volume have been often reported as statistica
296 , age, sex, and IQ were important sources of cerebellar volume variability, although independent of a
299 tocol describes a novel and rapid method for cerebellar window construction that can be set up in und
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