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1                                              SBMA embryonic motor neurons also displayed dysfunctiona
2                                              SBMA is a disease with high unmet clinical need.
3                                              SBMA is caused by CAG repeat expansions in the androgen
4                                              SBMA is caused by CAG-polyglutamine (polyQ) repeat expan
5                                              SBMA is caused by expansions of a polyglutamine tract in
6                                              SBMA is caused by polyglutamine repeat expansions in the
7                                              SBMA is characterized by progressive muscle atrophy of b
8                                              SBMA is due to an androgen receptor containing a polyglu
9                                              SBMA is one of nine polyQ diseases in which polyQ expans
10                                              SBMA patients exhibit myopathic features, suggesting a r
11                                              SBMA patients with more than 70 CAGs have never been obs
12 oding for a wild-type (19 CAG repeats) and a SBMA mutant androgen receptor (52 CAG repeats) were tran
13 s a valuable reagent for the production of a SBMA mouse.
14 o permit generation of mice that will show a SBMA phenotype within their life span, we decided to obt
15 nal activity and prevents many (but not all) SBMA-associated symptoms in this mouse model.
16 offering a novel treatment paradigm for ALS, SBMA, SMA, and related disorders.
17                 Soy-based meat alternatives (SBMA) are becoming increasingly popular, but it is uncle
18  SBMA symptoms, ASC-J9 treatment ameliorated SBMA symptoms by decreasing AR-97Q aggregation and incre
19  prevented toxicity and AR aggregation in an SBMA cell model and rescued primary SBMA motor neurons f
20 ate IRs were identified at the HD, SCA-7 and SBMA loci.
21 e cases, the molecular basis of many SMA and SBMA-like syndromes and most ALS cases is unknown.
22 cord and sciatic nerve of wild-type (WT) and SBMA mice at various stages of disease progression.
23  muscle-specific biomarkers useful to assess SBMA progression in mice and patients in response to pha
24 pment of spinal and bulbar muscular atrophy (SBMA or Kennedy disease).
25 ) causes spinal and bulbar muscular atrophy (SBMA) and is associated with misfolded and aggregated sp
26          Spinal and bulbar muscular atrophy (SBMA) impairs motor function in men and is linked to a C
27          Spinal and bulbar muscular atrophy (SBMA) is a hereditary neuromuscular disorder caused by C
28          Spinal and bulbar muscular atrophy (SBMA) is a heritable neurodegenerative disease caused by
29          Spinal and bulbar muscular atrophy (SBMA) is a motor neuron disease caused by polyglutamine
30          Spinal and bulbar muscular atrophy (SBMA) is a motor neuron disease caused by the expansion
31                Spinobulbar muscular atrophy (SBMA) is a neurodegenerative disease caused by expansion
32          Spinal and bulbar muscular atrophy (SBMA) is a neurodegenerative disease caused by the expan
33          Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular degenerative disease caused by
34          Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease caused by polyglutamine
35          Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease characterized by the lo
36          Spinal and bulbar muscular atrophy (SBMA) is a progressive neurodegenerative disease caused
37          Spinal and bulbar muscular atrophy (SBMA) is a progressive neuromuscular disease caused by p
38              Spinal bulbar muscular atrophy (SBMA) is a progressive, late onset neuromuscular disease
39 X-linked spinal and bulbar muscular atrophy (SBMA) is a rare form of motor neuron degeneration linked
40 X-linked Spinal and Bulbar Muscular Atrophy (SBMA) is a rare, late-onset neuromuscular disease caused
41          Spinal and bulbar muscular atrophy (SBMA) is a slowly progressing neuromuscular disease caus
42          Spinal and bulbar muscular atrophy (SBMA) is an inherited neuromuscular disorder caused by a
43 X-linked spinal and bulbar muscular atrophy (SBMA) is an inherited neuromuscular disorder characteriz
44                Spinobulbar muscular atrophy (SBMA) is an X-linked disease characterized by degenerati
45          Spinal and bulbar muscular atrophy (SBMA) is an X-linked motoneuron disease due to a CAG tri
46          Spinal and bulbar muscular atrophy (SBMA) is an X-linked, adult-onset neuromuscular conditio
47 X-linked spinal and bulbar muscular atrophy (SBMA) is caused by a CAG repeat expansion in the first e
48                Spinobulbar muscular atrophy (SBMA) is caused by CAG expansions in the androgen recept
49          Spinal and bulbar muscular atrophy (SBMA) is caused by polyglutamine expansion in the androg
50 X-linked spinal and bulbar muscular atrophy (SBMA) is characterized by adult-onset muscle weakness an
51          Spinal and bulbar muscular atrophy (SBMA) is characterized by loss of motoneurons and sensor
52          Spinal and bulbar muscular atrophy (SBMA) is one of a growing number of neurodegenerative di
53          Spinal and bulbar muscular atrophy (SBMA) is one of eight inherited neurodegenerative diseas
54          Spinal and bulbar muscular atrophy (SBMA) results from a CAG repeat expansion within the and
55 esponsible for spinobulbar muscular atrophy (SBMA) that leads to selective loss of lower motor neuron
56 leads to spinal and bulbar muscular atrophy (SBMA), a neurodegenerative disease characterized by lowe
57 X-linked spinal and bulbar muscular atrophy (SBMA), a neuromuscular disorder caused by expansion of a
58 oach for spinal and bulbar muscular atrophy (SBMA), a polyglutamine disorder that affects the motor n
59 X-linked spinal and bulbar muscular atrophy (SBMA), a repeat disorder caused by polyglutamine-expande
60          Spinal and bulbar muscular atrophy (SBMA), an adult-onset neurodegenerative disease that aff
61  disease spinal and bulbar muscular atrophy (SBMA), an adult-onset, slowly progressive motor neuron d
62 ) causes spinal and bulbar muscular atrophy (SBMA), an X-linked neuromuscular disease that is fully m
63 X-linked spinal and bulbar muscular atrophy (SBMA), and amyotrophic lateral sclerosis (ALS).
64 rosis (ALS), spinal-bulbar muscular atrophy (SBMA), and spinal muscular atrophy (SMA).
65 ted with spinal and bulbar muscular atrophy (SBMA), and the nuclear protein PTIP (Pax Transactivation
66 ncluding Spinal and Bulbar Muscular Atrophy (SBMA), Huntington's disease (HD), DentatoRubral and Pall
67 ncluding spinal and bulbar muscular atrophy (SBMA), Huntington's disease (HD), spinocerebellar ataxia
68 ch as X-linked spinobulbar muscular atrophy (SBMA), it is unknown whether the toxic form of the prote
69          Spinal and bulbar muscular atrophy (SBMA), or Kennedy's disease, is a late-onset motor neuro
70       In spinal and bulbar muscular atrophy (SBMA), polyQ expansion within the androgen receptor (AR)
71              Spinal bulbar muscular atrophy (SBMA), the first identified CAG-repeat expansion disorde
72 ) causes spinal and bulbar muscular atrophy (SBMA).
73 netic disorder spinobulbar muscular atrophy (SBMA).
74 or (AR) causes spinobulbar muscular atrophy (SBMA).
75 dividuals with spinobulbar muscular atrophy (SBMA).
76 icity in spinal and bulbar muscular atrophy (SBMA).
77 hy (DM), spinal and bulbar muscular atrophy (SBMA, also known as Kennedy disease), Huntington disease
78          Spinal and bulbar muscular atrophy (SBMA, also known as Kennedy's disease) is one of nine ne
79          Spinal and bulbar muscular atrophy (SBMA, Kennedy's disease) is one of a group of progressiv
80 X-linked spinal and bulbar muscular atrophy (SBMA; Kennedy's disease) is a polyglutamine (polyQ) dise
81  axonal transport in motoneurons affected by SBMA.
82 uclei in the presence of its ligand to cause SBMA.
83 ne with 97 CAG expansions and characteristic SBMA-like neurogenic phenotypes.
84 , symptomatic men with genetically confirmed SBMA.
85 rid system and nor did constructs containing SBMA or DRPLA with normal or expanded polyglutamine trac
86 ncover a crucial role for NLK in controlling SBMA toxicity and reveal a novel avenue for therapy deve
87   The observed pathology of NMJs in diseased SBMA mice is likely the morphological correlates of defe
88 euron-specific therapeutic interventions for SBMA.
89 of polyglutamine-expanded AR is required for SBMA, we also discovered, using cell models of SBMA, tha
90 utant androgen receptor (AR) responsible for SBMA in cell, fly and mouse models.
91 mulation is a novel therapeutic strategy for SBMA.
92 tant AR was necessary but not sufficient for SBMA.
93 rowth hormone is a potential therapeutic for SBMA.
94 sible benefit from mitochondrial therapy for SBMA.
95 in motor neuron precursor cells derived from SBMA patient stem cells, suggesting that Chmp7 may play
96 ling of primary embryonic motor neurons from SBMA mice.
97                                 Furthermore, SBMA muscle prominently features dysregulated calcium fl
98                                           In SBMA the polymorphic trinucleotide CAG repeat in exon 1
99                                           In SBMA, the nucleus is further implicated by the critical
100 a new pathogenic pathway that is affected in SBMA and results in compromised FAT.
101 al role for muscle expression of polyQ-AR in SBMA and suggest muscle-directed therapies as effective
102  may shed light on the pathogenic cascade in SBMA and other motor neuron diseases.
103 uisite for mutant AR-induced cytotoxicity in SBMA, reveal a compelling mechanistic link between ligan
104 t underlie the neuromuscular degeneration in SBMA and provide alternative therapeutic targets.
105 ls, the role of motor neuron degeneration in SBMA has not been rigorously investigated.
106 ction that produces neuronal degeneration in SBMA.
107 egulation occurs early during development in SBMA motor neurons.
108 al a novel avenue for therapy development in SBMA.
109 es evidence for mitochondrial dysfunction in SBMA cell and animal models, either through indirect eff
110  may critically mediate motor dysfunction in SBMA, but the site(s) where AR disrupts transport is unk
111 echanisms by which NLK exerts its effects in SBMA.
112          Furthermore, genes were enriched in SBMA motor neurons in several key pathways including p53
113 e examined the role of normal AR function in SBMA by crossing a highly representative AR YAC transgen
114 motor neurons, suggesting a role for NF-H in SBMA neuronal dysfunction.
115 , features that have both been implicated in SBMA; however, the extent to which altered AR transcript
116 on- a unique form of the N/C-interaction- in SBMA.
117 e option for pharmacological intervention in SBMA and potentially other polyglutamine diseases.
118 arget tissue for therapeutic intervention in SBMA.
119 tial targets for therapeutic intervention in SBMA.
120 ntial target for therapeutic intervention in SBMA.
121 sing target for therapeutic interventions in SBMA.
122  the requirement for nuclear localization in SBMA neurotoxicity, namely the lack of mutant AR removal
123 the importance of AR nuclear localization in SBMA pathogenesis; therefore, in this study, we sought t
124 d PRMT6 attenuates disease manifestations in SBMA mice.
125 l, this work identifies a novel mechanism in SBMA pathology that may represent a novel target for the
126 le reactivation as a pathogenic mechanism in SBMA.
127 ests that they underlie neurodegeneration in SBMA.
128 ed by neuromuscular disease, whether NMJs in SBMA are similarly affected by disease is not known.
129  To clarify the importance of the nucleus in SBMA, we genetically manipulated the nuclear localizatio
130 uncation fragment, as such peptides occur in SBMA patients and mouse models.
131 oxidative metabolic alterations occurring in SBMA muscles and induced hypertrophy of both glycolytic
132  vivo in spinal cord before symptom onset in SBMA mice, and crucially in motor neuron precursor cells
133  in amelioration of the disease phenotype in SBMA mice by restoring polyQ AR-dysregulated transcripti
134 7 knockdown suppressed disease phenotypes in SBMA and spinocerebellar ataxia type 3 (SCA3) fly models
135  ubiquitin-proteasome system is preserved in SBMA models.
136 d kinase plays more than a bystander role in SBMA-vulnerable postmitotic cells.
137 ion plays a pathogenic or protective role in SBMA.
138 a mild rescue in function and muscle size in SBMA mice, while genetic deletion of the gene encoding M
139 ed the effect of beta-agonist stimulation in SBMA myotube cells derived from mice and patients, and i
140 uld be a promising pharmacological target in SBMA.
141 ay serve as potential therapeutic targets in SBMA.
142 etylation as powerful therapeutic targets in SBMA.
143 ion in SBMA cells and suppresses toxicity in SBMA flies, and a preclinical approach based on miRNA-me
144 xpanded androgen receptor transactivation in SBMA cells and suppresses toxicity in SBMA flies, and a
145 ide on the progression of muscle weakness in SBMA, although there were secondary indications of both
146                                  Although KD/SBMA has been thought of as a motor neuron disease, rece
147 y's disease/spinobulbar muscular atrophy (KD/SBMA) through poorly defined cellular mechanisms.
148 ce for disordered cellular respiration in KD/SBMA skeletal muscle.
149 Is), derived from sulfobetaine methacrylate (SBMA), of different topologies: bottlebrush (BB-PSBMA) a
150  order to understand factors that may modify SBMA disease progression.
151 romotes disease pathogenesis across multiple SBMA model systems.
152                         We compared multiple SBMA NPC lines and documented the metabolic and autophag
153  in the sciatic nerve of adult WT and mutant SBMA mice demonstrated no overt axonal transport deficit
154                  Ultrastructural analysis of SBMA mice revealed a block in autophagy pathway progress
155 ith 112 glutamines reproduce many aspects of SBMA, including slowly progressive, gender-specific moto
156                    To elucidate the basis of SBMA, we expressed N-terminal truncated AR in motor neur
157 thologies described here, the development of SBMA therapeutics that preserve this activity may be des
158 of multiple pathways leads to development of SBMA.
159 ntranuclear inclusions (NIIs), a hallmark of SBMA and the other polyglutamine diseases.
160 export in the pathological manifestations of SBMA.
161 y underly neurite defects in a cell model of SBMA and identify senescence as a pathway implicated in
162 xamined axonal transport in a mouse model of SBMA recapitulating many aspects of the human disease.
163 e the creation of an inducible cell model of SBMA that reproduces this important characteristic of di
164                          In a mouse model of SBMA, evidence for DNA damage is detected in muscle cell
165    Using a differentiated PC12 cell model of SBMA, we identified dysfunction of polyQ-expanded AR in
166 length androgen receptor (AR) mouse model of SBMA.
167 nd a myogenic transgenic (TG) mouse model of SBMA.
168 ndent AR activation in a Drosophila model of SBMA.
169  of NMJs was examined in two mouse models of SBMA, a myogenic model that overexpresses wildtype andro
170 MA, we also discovered, using cell models of SBMA, that it is insufficient for both aggregation and t
171 induced toxicity in cell and mouse models of SBMA.
172 gregation and cytotoxicity in cell models of SBMA.
173  neurodegeneration in cell and fly models of SBMA.
174 uscle cells) and in vivo in animal models of SBMA.
175 o such global disruption occurs in models of SBMA; rather, AR-specific mechanisms, including reduced
176 anner specifically in the skeletal muscle of SBMA patients and mice.
177 is >4 oz SBMA patty and similarly to 8 oz of SBMA.
178 o play a pivotal role in the pathogenesis of SBMA using mouse models, the role of motor neuron degene
179 als a role for ligand in the pathogenesis of SBMA.
180 ay a significant role in the pathogenesis of SBMA.
181 ces both muscle and spinal cord pathology of SBMA mice.
182 ical role for USP7 in the pathophysiology of SBMA and suggest a similar role in SCA3 and Huntington's
183 s by consumption of 1 or two 4 oz patties of SBMA with 4 oz (80% protein/20% fat) beef.
184  which develop many of the motor symptoms of SBMA.
185  therapeutic target for treating symptoms of SBMA.
186 s to be tested in other cell types target of SBMA (i.e. muscle cells) and in vivo in animal models of
187 uperoxide dismutase-2 in affected tissues of SBMA knock-in mice.
188 ons holds great promise for the treatment of SBMA and other AR-related conditions; however, how this
189                                 Treatment of SBMA knock-in mice with clenbuterol, which was started a
190 ) and explore its therapeutic effects in our SBMA mouse model that harbors a mutant AR gene with 97 C
191 n the increase following consumption of 4 oz SBMA (P = 0.021; 0.003 +/- 0.010%/h) but not 8 oz SBMA (
192 beef (4 oz), single SBMA (4 oz), or two 4 oz SBMA patties (8 oz).
193 uscle and whole-body protein synthesis >4 oz SBMA patty and similarly to 8 oz of SBMA.
194 (P = 0.021; 0.003 +/- 0.010%/h) but not 8 oz SBMA (P = 0.454; 0.013 +/- 0.016%/h).
195 on in an SBMA cell model and rescued primary SBMA motor neurons from 5alpha-dihydrotestosterone-induc
196 FEB function to impair autophagy and promote SBMA pathogenesis.
197                                 The PTFEMA-r-SBMA membrane exhibits exceptional permeability and sele
198 -random-sulfobetaine methacrylate) (PTFEMA-r-SBMA) on the sensor surface using electrospray.
199 al or chemical castration was used to reduce SBMA symptoms, ASC-J9 treatment ameliorated SBMA symptom
200  remarkably, loss of one copy of Nlk rescues SBMA phenotypes in mice, including extending lifespan.
201  consumption of a single beef (4 oz), single SBMA (4 oz), or two 4 oz SBMA patties (8 oz).
202                                   We studied SBMA in Drosophila using an N-terminal fragment of the h
203 n synaptic muscle regions of pre-symptomatic SBMA mice, while extrasynaptic muscle features a coordin
204 ession in skeletal muscle of pre-symptomatic SBMA mice, with a focus on transcriptional changes at th
205                    Our studies indicate that SBMA disease pathogenesis, both in the nervous system an
206                 These findings indicate that SBMA pathogenesis is mediated by misappropriation of nat
207 atient-derived muscle biopsies, we show that SBMA mice in the presymptomatic stage develop a respirat
208                     Our results suggest that SBMA motor neuronopathy involves altered expression of V
209 rticularly prominent in cells expressing the SBMA androgen receptor.
210 ce of an expanded polyglutamine tract in the SBMA androgen receptor appears to enhance the production
211 AR canonical transcriptional activity in the SBMA pathologies described here, the development of SBMA
212 n order to investigate the properties of the SBMA androgen receptor in neuronal cells, cDNAs coding f
213 reduction in transcriptional activity of the SBMA mutant as compared with wild-type androgen receptor
214  a novel therapeutic strategy to prevent the SBMA phenotype while retaining AR transcriptional functi
215 ptor translocated to the nucleus whereas the SBMA androgen receptor was mainly localized in the cytop
216  and endocrine-reproductive features of this SBMA mouse model, as AR100Tfm mice displayed accelerated
217 ted several motor deficiencies in transgenic SBMA mice.
218 injection of ASC-J9 into AR-polyQ transgenic SBMA mice markedly improved disease symptoms, as seen by
219 at hormone-based therapies designed to treat SBMA patients, even with advanced disease, are likely to
220 peutic and preventative approach to treating SBMA, by disrupting the interaction between AR and AR co
221 to polyQ-expanded androgen receptor triggers SBMA through a combination of toxic gain-of-function and
222        Although the mechanisms that underlie SBMA remain unclear, defective axonal transport has been
223 ver cell-type-specific mechanisms underlying SBMA and to distinguish gain- from loss-of-function prop
224 , the exact pathogenic mechanisms underlying SBMA remain elusive.
225         The pathogenic mechanisms underlying SBMA remain unknown, but recent experiments show that in
226                                        Using SBMA AR113Q mice we analyzed proteotoxic stress-induced
227                                        Using SBMA as a model, we explored the relationship between pr
228                                     In vivo, SBMA transgenic mice overexpressing a muscle-specific is
229 y underlie motor impairments associated with SBMA.
230 tially improved motor function compared with SBMA mice.
231 ctase inhibitor dutasteride in patients with SBMA, and to identify outcome measures for use in future
232 ransgenic mice and tissue from patients with SBMA.
233 ntial therapeutic strategy for patients with SBMA.

 
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