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1 nts are useful for developing an aS-specific chemical chaperone.
2 ns lacking polyphosphate, a newly recognized chemical chaperone.
3 eticulum stress inhibitor, ammonia sink, and chemical chaperone.
4 t-containing nAChRs, possibly by acting as a chemical chaperone.
5 n and markers of ER stress are reversed by a chemical chaperone.
6 inase and could be inhibited by an exogenous chemical chaperone.
7 rinated nondetergent surfactants that act as chemical chaperones.
8 for therapeutic purposes with orally active chemical chaperones.
9 l and targetable to the plasma membrane with chemical chaperones.
10 gical correction of the processing defect by chemical chaperones.
11 egradation by using proteasome inhibitors or chemical chaperones.
12 mplications for the biological roles of such chemical chaperones.
17 Finally, we showed that administration of chemical chaperone 4-phenylbutyrate or genetic inhibitio
18 of Tat- or GFAP-induced UPR/ER stress by the chemical chaperone 4-phenylbutyrate significantly allevi
19 of miR-708 was blocked by treatment with the chemical chaperone 4-phenylbutyrate, uncovering the invo
20 Treatment of db/db mouse islets with the chemical chaperone 4-phenylbutyric acid partially restor
23 ment of MHV-A59-infected astrocytes with the chemical chaperone 4-sodium phenylbutyrate increased ERp
24 t the patient's fibroblasts treated with the chemical chaperones 4-phenylbutiric acid or curcumin inc
26 were observed with a structurally unrelated chemical chaperone, 4-phenylbutyric acid (100 mg.kg(-)(1
27 e pathobiology of these lesions, we employed chemical chaperone, 4-phenylbutyric acid (4-PBA) which a
33 macromolecular complexes, it is considered a chemical chaperone and has found numerous practical appl
34 nt was reversed using an ER stress-relieving chemical chaperone and via macrophage-specific knockdown
40 athogenicity of D1080N substitution and that chemical chaperones are therapeutic candidates for the m
43 ethylsulfoxide (DMSO), which we refer to as 'chemical chaperones' because of their influence on prote
44 eins are misfolded, but functional, and that chemical chaperones both correct the trafficking and fol
45 are small-molecule compounds that can act as chemical chaperones by altering the environment in a cel
46 tic underpinnings of concentration-dependent chemical chaperoning by ATP against prion-like transmiss
50 of ER stress by genetic deletion of CHOP or chemical chaperone conferred a resistance to the LPS-ind
51 escue provides a specific mechanism by which chemical chaperones could be developed for the correctio
52 CB4(S320F) and ABCB4(A953D), suggesting that chemical chaperones could be exploited for therapeutic b
54 portantly, treatment of patient cells with a chemical chaperone decreased intracellular COL4A2 levels
56 ilizing the active conformation of MKP3, the chemical chaperone dimethyl sulfoxide was able to mimic
57 e use of 4-phenylbutyric acid (4-PBA), an ER chemical chaperone, diminished ER stress markers and abo
58 Treatment with tauroursodeoxycholic acid, a chemical chaperone drug that functions as an endoplasmic
59 n aggregation is an important determinant of chemical chaperone efficiency under endogenous expressio
63 -conjugated bile acids (TBA) facilitates TBA chemical chaperone function and alleviates endoplasmic r
67 degradation is blunted by treatment with the chemical chaperone glycerol, which retains SM N100-GFP i
73 ew study highlights a critical role for this chemical chaperone in energy-independent maintenance of
74 tance, detailed studies on the role of these chemical chaperones in modulating structure and dimensio
75 stration of sodium 4-phenylbutyrate (PBA), a chemical chaperone, increased protein stability, enzymat
76 roblasts at a permissive temperature or with chemical chaperones increases cellular Hex activity by i
78 ization of the dissociated PTS1 subunit with chemical chaperones inhibited toxin export to the cytoso
80 g the efficiency of tyrosinase folding using chemical chaperones led to a reduction in the level of s
81 cells initially accumulate both protein and chemical chaperones, like Hsp104 and trehalose, respecti
82 Attenuating ER stress with clinically used chemical chaperones may be a novel therapeutic strategy
83 propriate specificity and affinity acting as chemical chaperones may find application for increasing
84 ailure in Wolfram syndrome and indicate that chemical chaperones might have therapeutic relevance und
87 ogical manipulation of this pathway by using chemical chaperones offers a therapeutic option for trea
90 Reduction of ER stress by treatment with chemical chaperones or overexpression of ER chaperone pr
93 l taurine, an amino acid that functions as a chemical chaperone/osmolyte and enhances cellular antiox
95 in-1 expression, while administration of the chemical chaperone PBA to ob/ob mice led to amelioration
96 from protein misfolding, as treatment with a chemical chaperone permits it to exit the endoplasmic re
97 ral treatment of Col4a1 mutant mice with the chemical chaperone phenyl butyric acid (PBA), which redu
101 d the degradation mechanisms, we tested some chemical chaperones previously used for treating CFTR F5
103 h a US Food and Drug Administration-approved chemical chaperone resulted in a decreased collagen intr
104 we show that mitigation of ER stress with a chemical chaperone results in marked protection against
105 Conversely, reduction of ER stress with a chemical chaperone significantly protected against both
107 ro studies reported a positive effect of the chemical chaperone sodium 4-phenylbutyrate (4-PBA) on mu
110 f hyperostosis that can be suppressed with a chemical chaperone, sodium 4-phenobutyrate (4-PBA).
111 of mice carrying the I4895T mutation with a chemical chaperone, sodium 4-phenylbutyrate (4PBA), redu
112 telet aggregation, while clinically approved chemical chaperone, sodium 4-phenylbutyrate reduced the
113 by co-treatment with either of two different chemical chaperones, sodium 4-phenylbutyrate and taurour
114 ess protectants, but also serve as effective chemical chaperones stabilizing protein functionality.
115 munoglobulin protein (BiP) or treatment with chemical chaperones strengthens the oscillation amplitud
116 s a novel target to ameliorate OI phenotype; chemical chaperones such as 4PBA may be, alone or in com
117 ght CBS mutants expressed in the presence of chemical chaperones such as ethanol, dimethyl sulfoxide,
118 y shown that Hmg2p is strongly stabilized by chemical chaperones such as glycerol, which stabilize mi
119 The above in vitro results offer the use of chemical chaperones such as TMAO as an approach to mitig
121 ns, protecting against ER stress using small chemical chaperones, such as 4-phenylbutyrate and taurou
125 cation in mice that have been treated with a chemical chaperone, supporting the connection between im
127 ent of cells expressing C321R-LAMB2 with the chemical chaperone taurodeoxycholic acid (TUDCA), which
129 ced chaperone response and the orally active chemical chaperones tauroursodeoxycholate (TUDCA) and 4-
130 1RjK) heterozygous pregnant females with the chemical chaperone tauroursodeoxycholic acid alleviated
133 proapoptotic UPR, whereas application of the chemical chaperones tauroursodeoxycholic acid or 4-pheny
134 is study is to examine the efficacy of an ER chemical chaperone, tauroursodeoxycholic acid (TUDCA), i
136 wild-type phenotype with the addition of the chemical chaperone, tauroursodexycholic acid (TUDCA).
140 hermore, following treatment with a panel of chemical chaperones (thapsigargin, glycerol or sodium 4-
142 We propose that polyphosphate acts as a chemical chaperone that helps refold MetA and/or may sti
143 reatment with 4-phenylbutyrate, a well-known chemical chaperone that improves trafficking of several
144 ministration of tauroursodeoxycholic acid, a chemical chaperone that inhibits ER stress, significantl
146 he defects are not reversed with glycerol, a chemical chaperone that rescues channel function in some
148 f these conditions has led to the search for chemical chaperones that can slow, arrest or revert dise
149 UDCA), a bile acid derivative that acts as a chemical chaperone to enhance protein folding and amelio
151 ian cells and degraded but can be rescued by chemical chaperones to function as plasma membrane water
155 ort here that sulfonylureas also function as chemical chaperones to rescue K(ATP) channel trafficking
156 cancer: from the structure-guided design of chemical chaperones to restore the function of conformat
157 utic approaches to age-related cataracts use chemical chaperones to solubilize HMW aggregates, while
162 onse involves a dramatic accumulation of the chemical chaperone trehalose and induction of trehalose-
166 le to demonstrate that administration of the chemical chaperone TUDCA helped to maintain lymphocyte h
167 R stress in vitro using dexamethasone or the chemical chaperones TUDCA and 4PBA attenuated MMP9 expre
168 ent with 4-phenylbutyric acid, a nonspecific chemical chaperone used in other protein-folding disorde
169 s in the living cell, the in vitro action of chemical chaperones was highly specific for Hmg2p and co
170 that changes similar to those observed with chemical chaperones were brought about by alteration of