ライフサイエンスコーパス: proteostasis

1 deregulated nutrient sensing and defects in proteostasis).

2 etal muscle protein synthesis and breakdown (proteostasis).

3 al roles in maintaining protein homeostasis (proteostasis).

4 key role in maintaining protein homeostasis (proteostasis).

5 jectories are significantly impacted by host proteostasis.

6 degradation of misfolded proteins to restore proteostasis.

7 Protein chaperones play a critical role in proteostasis.

8 is a key regulator of podocyte foot process proteostasis.

9 g neuroprotective effects involving improved proteostasis.

10 NA, indicative of a profound perturbation to proteostasis.

11 f sequence-specific signals in mitochondrial proteostasis.

12 rone and degradation pathways promoting mHtt proteostasis.

13 nship between the TRC40 pathway and cellular proteostasis.

14 various signaling pathways or during general proteostasis.

15 ibutions of IR and IGF1R signaling to muscle proteostasis.

16 ivo to survive multiple stressors related to proteostasis.

17 creased protein aggregation and altered cell proteostasis.

18 to impaired proteasome function and altered proteostasis.

19 to the cytosol and is essential to maintain proteostasis.

20 hat may have specialized roles in regulating proteostasis.

21 a posttranslational modification to maintain proteostasis.

22 and ubiquitination to ciliary signalling and proteostasis.

23 associated with stress or general decline in proteostasis.

24 proper neddylation levels for CRL-dependent proteostasis.

25 insight into disease mechanisms and cellular proteostasis.

26 ess the potential of the chemical biology of proteostasis.

27 e their contribution to maintaining cellular proteostasis.

28 aintain genome stability by assuring nuclear proteostasis.

29 which UPR activation regulates extracellular proteostasis.

30 ins by the proteasome system is critical for proteostasis.

31 l protein synthesis is paramount to maintain proteostasis.

32 hich permits simultaneous stress sensing and proteostasis.

33 y reduce ER protein folding load and restore proteostasis.

34 s to PERK regulation of pancreatic beta-cell proteostasis.

35 defective mitochondrial import and impaired proteostasis.

36 recently been associated with alterations in proteostasis.

37 nvolved in coordinating ER and extracellular proteostasis.

38 ROS) leads to perturbations in mitochondrial proteostasis.

39 ular processes, including the maintenance of proteostasis.

40 cal processes that are critical for cellular proteostasis.

41 ochondria in metabolism, ROS regulation, and proteostasis.

42 ay be physiologically relevant in ER luminal proteostasis.

43 chaperones with important roles in cellular proteostasis.

44 cell type-specific proteomes, and coordinate proteostasis across tissues.

45 Cellular proteostasis alterations resulted in cardiomyopathy char

46 new insights into the physiology of cellular proteostasis and a rational basis for developing effecti

47 ulated functions in the heat-shock response, proteostasis and ageing.

48 orylation regulates not only cell-autonomous proteostasis and amino acid metabolism, but also affects

49 ulates the ER stress response and elicits ER proteostasis and autophagy machinery homeostasis in huma

50 e heat shock response (HSR) is essential for proteostasis and cellular health.

51 It is unclear how these events control ER proteostasis and cellular health.

52 ged as a key mechanism required for membrane proteostasis and cellular signaling.

53 d lysosomal protein degradation for cellular proteostasis and clearance of aggregated proteins, these

54 0 has an unexpected negative effect on Abeta proteostasis and cognition in APP mouse models demonstra

55 e reviewed here in the context of sub-system proteostasis and complex adaptive systems theory.

56 that AL LC expression alters cell growth and proteostasis and confers PI sensitivity.

57 ffect also included an increase in lysosomal proteostasis and enhanced clearance of lysosomal storage

58 erated and whether the process regulates VHL proteostasis and function are unknown.

59 We analyzed VHL-R167Q proteostasis and function at normoxia, at hypoxia with d

60 udy reveals a novel mechanism regulating VHL proteostasis and function, which is significant for iden

61 capacity, leading to impaired mitochondrial proteostasis and function.

62 sential for the maintenance of mitochondrial proteostasis and health.

63 s underlying the regulation of mitochondrial proteostasis and how those mechanisms change with age is

64 tent and specific correction of F508del-CFTR proteostasis and in synergy with pharmacochaperones.

65 therapies, whether lowering ammonia restores proteostasis and increases muscle mass is unknown.

66 conceptually new role for H2O2 signaling in proteostasis and lifespan control and shed new light on

67 a model in which VAP-ligand binding couples proteostasis and lipid homeostasis leading to observed p

68 F signaling has profound effects on neuronal proteostasis and maintenance of cell morphology in vivo.

69 es have broad implications for CRL-dependent proteostasis and mechanisms of E3-mediated UB ligation.

70 tracycline derivative-disturbs mitochondrial proteostasis and metabolic activity, and induces widespr

71 and that enhanced disaggregases can restore proteostasis and mitigate neurodegeneration.

72 ut formerly unrecognized, branch of neuronal proteostasis and mitochondrial quality control, which, w

73 adation, autophagy and mitophagy to maintain proteostasis and mitochondrial quality.

74 e to rid cells of neurotoxic components when proteostasis and organelle function are challenged.

75 ltiple points to coordinate their actions in proteostasis and organelle homeostasis.

76 xenobiotic stress response so as to maintain proteostasis and prepare for possible infection.

77 mechanism that helps maintain intracellular proteostasis and promote cell survival during ER stress

78 escence, mitochondrial dysfunction, impaired proteostasis and reduced stress resistance.

79 ts interaction suggests a connection between proteostasis and RNA metabolism.

80 ein cleavage, and the cleavage regulates VHL proteostasis and subsequent function.

81 the oncogenic RAS-MEK signaling in guarding proteostasis and suppressing amyloidogenesis.

82 transcriptional program that is essential to proteostasis and survival under such conditions.

83 In particular, dysfunction of proteostasis and the resultant apoptotic death of neuron

84 s eliminate defective polypeptides to ensure proteostasis and to avoid the toxicity of protein aggreg

85 ination of misfolded proteins is crucial for proteostasis and to prevent proteinopathies.

86 tabolizes intracellular contents to maintain proteostasis and to produce energy during nutrient depri

87 in lysosomes, contributes to maintenance of proteostasis and to the cellular adaptation to stress.

88 on neuronal activity-mediated regulation of proteostasis and transcellular propagation of protein ag

89 uced protein turnover, increasingly abnormal proteostasis and, ultimately, faster onset of disease sy

90 ineries and networks of protein homeostasis (proteostasis) and stress resistance pathways as critical

91 directed more toward somatic maintenance and proteostasis, and away from cell growth and proliferatio

92 ctive autophagy, telomere attrition, altered proteostasis, and cell senescence.

93 1 that profoundly impacts stress resistance, proteostasis, and malignant growth.

94 ed jump and flight ability, disrupted muscle proteostasis, and severely perturbed IFM structure.

95 to be especially susceptible to failures in proteostasis, and this is now increasingly recognized as

96 eas failure to maintain protein homeostasis (proteostasis) appears to be a component of aging and a v

97 Imbalances in endoplasmic reticulum (ER) proteostasis are associated with etiologically-diverse d

98 od, abnormal protein aggregation and altered proteostasis are common features of sporadic and familia

99 ny details underlying the regulations of VHL proteostasis are unknown.

100 chondrial dynamics, and protein homeostasis (proteostasis) as fundamental regulators of stem cell fun

101 An imbalance of neuronal proteostasis, associated with protein misfolding and agg

102 membrane-anchored AAA+ enzyme that controls proteostasis at the inner membrane and intermembrane spa

103 pathway are unlikely attributed to defective proteostasis because up-regulation of protein synthesis

104 fy a lysosomal switch that enhances germline proteostasis before fertilization.

105 The UPR attempts to restore proteostasis but if unsuccessful drives affected cells t

106 een associated with a progressive decline of proteostasis, but how this process affects proteome comp

107 results suggest Hsp90 and p23 contribute to proteostasis by chaperoning mature factors through energ

108 rotein aggregates for degradation, and reset proteostasis by enveloping and clearing the aggregates.

109 ed the effect of chronic hyperinsulinemia on proteostasis by generating a time-resolved map of insuli

110 o-chlorobenzylidene)amino]guanidines restore proteostasis by interfering with eIF2alpha-P dephosphory

111 the cellular lipid landscape that disrupt ER proteostasis by interfering with the glycan trimming and

112 Notably, increasing mitochondrial proteostasis by pharmacologically and genetically target

113 ic acids directly participate in maintaining proteostasis by preventing protein aggregation.

114 Cells maintain proteostasis by selectively recognizing and targeting mi

115 rt the notion that cytosolic Fes1S maintains proteostasis by supporting the removal of toxic misfolde

116 h diverse cellular activities) that maintain proteostasis by unfolding aberrant and toxic proteins fo

117 mary, we demonstrate that targeting cellular proteostasis can inhibit norovirus replication, identify

118 Disruption of proteostasis can lead to the accumulation of protein agg

119 y regulated; changes in folding homeostasis (proteostasis) can disrupt many cellular processes and ha

120 s innate protective pathway, which maintains proteostasis, can be harnessed effectively to protect ol

121 here a novel response pathway that enhances proteostasis capacity and appears to act in parallel to

122 A decline of proteostasis capacity during aging leads to dysfunction

123 Enhancement of the cellular proteostasis capacity with small molecules has therefore

124 We consider different strategies to modulate proteostasis capacity, which may help develop urgently n

125 itochondria, while simultaneously increasing proteostasis capacity.

126 c hyponatremia induces severe alterations in proteostasis characterized by diffuse protein aggregatio

127 These findings indicate that proteostasis collapse serves as an intrinsic cue to caus

128 by the absence of sperm exhibit hallmarks of proteostasis collapse, including protein aggregation.

129 an illuminate the interplay between cellular proteostasis components and their distinct substrates.

130 cancer-subtype dependencies upon particular proteostasis components are relatively undefined.

131 stress, including oncogenic transformation, proteostasis components maintain homeostasis and prevent

132 oplasmic reticulum (ER) stress and disrupted proteostasis contribute to the pathogenesis of a variety

133 tal muscle, which is a loss of mitochondrial proteostasis, contributes to tissue dysfunction and nega

134 Regulated intracellular proteostasis, controlled in part by proteolysis, is esse

135 Our study suggests that manipulation of proteostasis could be an alternative approach to the tre

136 aggregates is a protective strategy to slow proteostasis decline during nematode aging.

137 Proteostasis depends on a network of molecular chaperone

138 findings support the oncogenic relevance of proteostasis deregulation in hematopoietic cells, and th

139 ects organisms from symptoms associated with proteostasis disorders, suggesting that protein clearanc

140 , may lead to defective protein homeostasis (proteostasis) due to hyperactivation of insulin-sensitiv

141 clients directly links ER and extracellular proteostasis during conditions of ER stress.

142 Reprogramming of the ER proteostasis environment through genetic activation of t

143 py the ATF6-mediated reprogramming of the ER proteostasis environment.

144 displaying chemically-controlled, divergent proteostasis environments.

145 inflammatory secretory phenotype, defects in proteostasis, epigenetic changes, deregulated nutrient s

146 ext-generation shRNA platform, we found that proteostasis factors, including chaperones and stress-re

147 otic demyelination might be a consequence of proteostasis failure on severe osmotic stress.

148 Proper maintenance of nuclear proteostasis has important implications in preserving ge

149 rs, but the relative roles of each in muscle proteostasis have not been fully elucidated.

150 Change in proteostasis, however, is not without repercussions.

151 both mitochondrial respiratory function and proteostasis in aged flies.

152 To understand the etiology of Abeta proteostasis in AMD, we delivered recombinant adeno-asso

153 hus, a lysosomal switch that enhances oocyte proteostasis in anticipation of fertilization may be con

154 is more important than IGF1R in controlling proteostasis in differentiated muscle.

155 release the neurohormone dopamine to promote proteostasis in epithelia.

156 workshop on "Malformed Protein Structure and Proteostasis in Lung Diseases" was to identify mechanist

157 highlights a previously overlooked role for proteostasis in maintaining cell survival in the absence

158 chanistic intersection of RNA processing and proteostasis in mediating neuroprotection.

159 a transcriptional program aimed at restoring proteostasis in mitochondria.

160 omplex interplay between innate immunity and proteostasis in neurodegenerative diseases, an interacti

161 of which are shown to disrupt mitochondrial proteostasis in other contexts-we hypothesized that fail

162 a central role in the loss of mitochondrial proteostasis in skeletal muscle ageing, as well as its b

163 nerves and/or muscle modifies mechanisms of proteostasis in skeletal muscle and plays a key role in

164 nvolved in coordinating ER and extracellular proteostasis in the presence and absence of ER stress.

165 To examine RC subunit proteostasis in vivo, we measured RC protein half-lives

166 We propose that VCP sustains sarcoplasmic proteostasis, in part, by controlling the integrity of a

167 hinery that manages proteome homeostasis, or proteostasis, in the cell.

168 s that adversely affect protein homeostasis (proteostasis), including extreme temperatures, toxins, a

169 rone, which plays a central role in cellular proteostasis, including quality control during protein r

170 Disturbance of endoplasmic reticulum (ER) proteostasis is a common feature of amyotrophic lateral

171 mental illness as a result of disruptions in proteostasis is an emerging theme in the study of schizo

172 Trans-NIH Geroscience Initiative and loss of proteostasis is associated with aging and age-related ch

173 This means of rebalancing proteostasis is conserved from yeast to humans.

174 terminants, the loss of proteostasis - where proteostasis is defined here as the maintenance of the p

175 role of pentraxins to maintain extracellular proteostasis is discussed.

176 Proteostasis is maintained by multiple cellular pathways

177 Proteostasis is one of the seven "pillars of aging resea

178 VHL proteostasis is regulated by multiple mechanisms includi

179 Loss of protein homeostasis (proteostasis) is a common feature of aging and disease t

180 Protein homeostasis (proteostasis) is inextricably tied to cellular health an

181 Maintenance of protein homeostasis, or proteostasis, is attained through precisely coordinated

182 Maintenance of protein homeostasis, or proteostasis, is crucial for organismal health.

183 r chaperone for maintenance of mitochondrial proteostasis, is highly expressed in glioblastoma patien

184 Protein homeostasis, or proteostasis, is required for mitochondrial function, bu

185 ls evoke an adaptive mechanism to restore ER proteostasis known as the unfolded protein response (UPR

186 olutionarily conserved regulator of cellular proteostasis linked to longevity in nematodes, but its b

187 We find that the proteostasis machine recognizes and sorts a client prote

188 The F508del-CFTR proteostasis machinery and its homeostatic regulation ar

189 respond to oxidative stress by up-regulating proteostasis machinery, but the direct activation of mam

190 Cellular protein homeostasis (proteostasis) maintains the integrity of the proteome an

191 Proteostasis maintenance of gamma-aminobutyric acid type

192 ular chaperones, is a key player in neuronal proteostasis maintenance.

193 ntrol, aggregate prevention, and, therefore, proteostasis maintenance.

194 inst a multi-functional ATPase essential for proteostasis maintenance.

195 ppression of genes essential for maintaining proteostasis; many are hub genes - involved in RNA proce

196 arly, interspecific comparisons suggest that proteostasis may be an important lifespan determinant in

197 sized that failure to maintain mitochondrial proteostasis may play a role in hypoxic injury.

198 nd that autophagy is an important endogenous proteostasis mechanism and an attractive target for ther

199 We term this mitochondria-mediated proteostasis mechanism MAGIC (mitochondria as guardian i

200 We hypothesized that host proteostasis mechanisms may be significant determinants

201 The results support the concept that "proteostasis" mechanisms, such as intracellular degradat

202 , the role of GAPDH protofibrils in neuronal proteostasis must be considered.

203 ential in all organisms and regulated by the proteostasis network (PN) and cell stress response pathw

204 The proteostasis network (PN) regulates protein synthesis, f

205 n aggregation is routinely suppressed by the proteostasis network (PN), a collection of macromolecula

206 ggregates that challenge the capacity of the proteostasis network (PN), increasing the risk for disea

207 We show evidence that a complex proteostasis network actively combats protein aggregatio

208 Hsf1 to flexibly integrate signals from the proteostasis network and cell signaling pathways.

209 additional inputs/factors in this underlying proteostasis network and demonstrates the utility of zeb

210 artnership between two key components of the proteostasis network and implicate autophagy defects in

211 olerance of the heart presumably through the proteostasis network and reinforces the critical role of

212 in the first experimental paradigm, cellular proteostasis network capacity and its dynamics are refle

213 client-based probes to quantify the cellular proteostasis network capacity in real time is highly des

214 Insufficient proteostasis network capacity is reflected by aggregate

215 transcriptional program augmenting cytosolic proteostasis network capacity, and in part by time-depen

216 tasis in these organelles is maintained by a proteostasis network containing protein chaperones, pept

217 A complex and adaptive proteostasis network coordinates these processes with mo

218 te to aggregate formation, components of the proteostasis network dictate the fate of protein aggrega

219 The proteostasis network has evolved to support protein fold

220 Targeting the proteostasis network in association to VPA treatment may

221 al to its function as a key component of the proteostasis network in cells.

222 tional role of specific components of the ER proteostasis network in the cellular changes associated

223 The cellular proteostasis network integrates the protein folding and

224 The proteostasis network is a highly conserved network of ce

225 The proteostasis network is decomposed into different module

226 ret this result to reflect the presence of a proteostasis network that can "sense" protein stability.

227 can result in non-oncogene addiction to the proteostasis network that can be exploited clinically.

228 these data identify ERdj5 as a member of the proteostasis network that regulates rod opsin biogenesis

229 ategies to target-specific components of the proteostasis network using small molecules and gene ther

230 nate up-regulation of proteins in the global proteostasis network, including ribosomal, proteasomal,

231 d depending on the targeted component of the proteostasis network.

232 challenges the cellular protein homeostasis (proteostasis) network capacity of cells by consuming cha

233 contain an extensive protein homeostasis (or proteostasis) network comprising molecular chaperones an

234 ated with an unbalanced protein homeostasis (proteostasis) network, which sensitizes cancer cells to

235 ting cancer subtype-specific dependencies on proteostasis networks to uncover unanticipated cancer vu

236 highlight the multidimensional nature of the proteostasis networks, which allow for coordinated prote

237 identify profound rearrangement in cellular proteostasis occurring very early on after loss of ATM i

238 t was predicted to preferentially govern the proteostasis of APP-C99.

239 he nuclear envelope, which in turn regulates proteostasis of certain inner KT components.

240 nslocation selectively impaired glycoprotein proteostasis of influenza as well as HIV and dengue viru

241 cement, represents a new strategy to restore proteostasis of misfolding-prone GABAA receptors and, th

242 n-dependent folding system also controls the proteostasis of other membrane proteins as CFTR and anth

243 haperone protein dHsc4/Hsc70 to maintain the proteostasis of PLIN2.

244 rone activities of Ranbp2 cyclophilin toward proteostasis of selective substrates and with novel ther

245 Furthermore, we implicate Rnf145 in proteostasis of the Nox2 complex by endoplasmic reticulu

246 endoplasmic reticulum lumen regulates normal proteostasis of the secretory pathway; they also support

247 Modulation of the proteostasis of VHL, especially missense point-mutated V

248 cosylation alters the protein homeostasis or proteostasis of wild-type (WT) and R345W F3 in ARPE-19 c

249 ent aspects of chaperone control of neuronal proteostasis on topics ranging from chaperone engineerin

250 sperm-secreted hormones re-establish oocyte proteostasis once fertilization becomes imminent.

251 suggesting that transcription errors affect proteostasis particularly in aging cells.

252 e studies allude to the existence of a novel proteostasis pathway that mechanistically links misfolde

253 logy, we explored the role of major cellular proteostasis pathways and mitochondrial proteases in FXN

254 Here we provide an overview of proteostasis pathways and their interplay (particularly

255 Here we discuss the major proteostasis pathways in light of recent research sugges

256 Targeting proteostasis pathways therapeutically remains an attract

257 d appears to act in parallel to well-studied proteostasis pathways.

258 nal remodeling of endoplasmic reticulum (ER) proteostasis pathways.

259 y disrupted in autophagy and in compensatory proteostasis pathways.

260 ve proteomics of clpt1 clpt2 plants showed a proteostasis phenotype similar to viable mutants in ClpP

261 ipid sorting and sensing proteins, LSS) with proteostasis regulation.

262 We probed the ER retention process using the proteostasis regulator 4-phenylbutyrate (4-PBA), which w

263 ing the mechanisms of action of F508del-CFTR proteostasis regulator drugs through an approach based o

264 ed a rationale for therapeutically targeting proteostasis regulators (e.g., HSP90), cancer-subtype de

265 l and physical interactions between VAPB and proteostasis regulators (ligands), including the unfolde

266 Whilst the induction of proteostasis-related genes was dependent on HSF1, the re

267 s and phosphorylation cascades might control proteostasis remains barely explored.

268 function, it has been assumed that synaptic proteostasis requires the ubiquitin-proteasome system (U

269 Ensuring cellular protein homeostasis, or proteostasis, requires precise control of protein synthe

270 e that SQRD-1 activity in H2S may coordinate proteostasis responses in multiple cellular compartments

271 stressors that disrupt protein homeostasis (proteostasis), resulting in protein misfolding and aggre

272 ies indicated by these recent discoveries in proteostasis science that will advance our molecular und

273 dock on cellular membranes thus performing a proteostasis sensor function.

274 Consistent with its role in defending proteostasis, Sephin1 attenuated the IRE1 branch of the

275 g mHtt structural properties to its neuronal proteostasis should inform new strategies for neuroprote

276 stability and folding and not by markers of proteostasis stress such as protein carbonylation and ag

277 s an enabler, allowing cancer cells to evade proteostasis stress triggered by oncogene activation.

278 13, the depletion of FGF13 elicits increased proteostasis stress, associated with the accumulation of

279 e that, as part of the in vivo extracellular proteostasis system, the plasminogen activation system m

280 ution, and into the potential design of host proteostasis-targeted antiviral therapeutics that are re

281 ionship between fatty acid metabolism and ER proteostasis that influences cell viability.

282 t mutation (F508del-CFTR) results in altered proteostasis, that is, in the misfolding and intracellul

283 bound to a large set of genes implicated in proteostasis, the acute-phase response, metabolism, and

284 systems of Escherichia coli work together in proteostasis: the recognition, sorting, folding, and dis

285 ts suggest that TRiC contributes to AML1-ETO proteostasis through specific interactions between the o

286 ation also directly influences extracellular proteostasis through the upregulation and secretion of t

287 nse (UPR) indirectly regulates extracellular proteostasis through transcriptional remodeling of endop

288 t modulates both longevity and mitochondrial proteostasis throughout life.

289 ort the relevance of enhancing mitochondrial proteostasis to delay amyloid-beta proteotoxic diseases,

290 Therefore, tumors exploit ClpXP-directed proteostasis to maintain mitochondrial bioenergetics, bu

291 nant state, and disrupting the fragile tumor proteostasis to promote amyloidogenesis may be a feasibl

292 clear that the capacity of cells to maintain proteostasis undergoes a decline during aging, rendering

293 Loss of proteostasis underlies ageing and neurodegeneration char

294 such as yeast Cur1 protein, play key role in proteostasis via tight control of partitioning and recyc

295 normal muscle mitochondrial structure and/or proteostasis vs. empty vector controls.

296 The improvement in proteostasis was attributed to a coordinate up-regulatio

297 conserved lifespan determinants, the loss of proteostasis - where proteostasis is defined here as the

298 t these cells suffer from a profound loss in proteostasis, which sensitizes them to the expression of

299 , describes new work in the area of neuronal proteostasis, with a specific focus on the roles and the

300 haperone protein responsible for maintaining proteostasis, yet how its structure translates into func