ライフサイエンスコーパス: phenylbutyrate

1 by the histone deacetylase inhibitor sodium phenylbutyrate.

2 of the histone deacetylase inhibitor sodium phenylbutyrate.

3 roxy-4-phenylbutyrate, and 2-hydroxy-4-oxo-4-phenylbutyrate.

4 entinostat, romidepsin, bufexamac and sodium phenylbutyrate.

5 ll SCFAs that are HDAC inhibitors, including phenylbutyrate.

6 administration of the molecular chaperone 4-phenylbutyrate.

7 butyl-1-methylxanthine, 2) BPDZ 154, or 3) 4-phenylbutyrate.

8 focused on testing repurposed drugs such as phenylbutyrate (2), valproic acid (3), riluzole (6), hyd

9 In vitro, chaperone drugs, such as 4-phenylbutyrate (4-PB), have been shown to partially corr

10 ve effect of the chemical chaperone sodium 4-phenylbutyrate (4-PBA) on mutant UMOD maturation.

11 eviously shown that the chemical chaperone 4-phenylbutyrate (4-PBA) promotes the maturation of ABCC6

12 The therapeutic peroxisome proliferator, 4-phenylbutyrate (4-PBA) was investigated in vitro and in

13 Treatment of mice with 4-phenylbutyrate (4-PBA), a chemical chaperon and a potent

14 tween SURF4 and SEC24A can be inhibited by 4-phenylbutyrate (4-PBA), a small molecule that occludes a

15 aperones tauroursodeoxycholate (TUDCA) and 4-phenylbutyrate (4-PBA), as well as the iron chelator cic

16 s C but little benefit from the folding drug phenylbutyrate (4-PBA), suggesting a tolerated level of

17 n process using the proteostasis regulator 4-phenylbutyrate (4-PBA), which we show targets COPII prot

18 ation for rescue by the chemical chaperone 4-phenylbutyrate (4-PBA).

19 e (NAC, a blocker of oxidative stress) and 4-phenylbutyrate (4-PBA, a blocker of endoplasmic reticulu

20 and chaperone drugs (ursodeoxycholic acid, 4-phenylbutyrate [4-PB]) were investigated.

21 n cellular coupling was strengthened using 4-phenylbutyrate (4PB).

22 Previous studies have shown that both 4-phenylbutyrate (4PBA) and trichostatin A (TSA) increase

23 es include histone deacetylase inhibitors, 4-phenylbutyrate (4PBA) and trichostatin A, and two small

24 Sodium 4-phenylbutyrate (4PBA) corrects trafficking of DeltaF508-

25 Sodium 4-phenylbutyrate (4PBA) improves the intracellular traffic

26 mutation with a chemical chaperone, sodium 4-phenylbutyrate (4PBA), reduces ER stress/UPR and improve

27 A chemical corrector, 4-phenylbutyrate (4PBA), restored LGI1(E383A) folding and

28 a known transcriptional regulator, sodium 4-phenylbutyrate (4PBA), will enable a greater fraction of

29 Similar to NaPA, sodium phenylbutyrate, a chemically synthesized precursor of Na

30 Treating Col4a1(+/G1344D) mice with 4-phenylbutyrate, a compound that promotes the trafficking

31 r treatment of PBD fibroblasts with sodium 4-phenylbutyrate, a human peroxisome proliferator, there w

32 Encouragingly, however, 4-phenylbutyrate, a pharmacological corrector, reduced L92

33 Encouragingly, however, 4-phenylbutyrate, a pharmacological corrector, reduced L92

34 that a low temperature eye mask and sodium 4-phenylbutyrate, a United States Food and Drug Administra

35 Supporting this, treatment with 4-phenylbutyrate, a well-known chemical chaperone that imp

36 jection for 5 to 21 days in combination with phenylbutyrate administered as a continuous intravenous

37 ermine whether L-ornithine and phenylacetate/phenylbutyrate (administered as the pro-drug phenylbutyr

38 However, phenylbutyrate administration decreases plasma branched-

39 rations, and previous research suggests that phenylbutyrate administration may increase leucine oxida

40 We investigated the effects of phenylbutyrate administration on whole-body protein meta

41 Prolonged phenylbutyrate administration reduced ureagenesis and th

42 Phenylbutyrate administration reduced ureagenesis by app

43 tration of the histone deacetylase inhibitor phenylbutyrate after onset of symptoms in a transgenic m

44 tly reduced by >50%; no effect was seen with phenylbutyrate alone.

45 lacetate or sodium phenylbutyrate, or sodium phenylbutyrate alone.

46 m phenylbutyrate-taurursodiol (3 g of sodium phenylbutyrate and 1 g of taurursodiol, administered onc

47 t with the slow-release behavior of glycerol phenylbutyrate and better overnight ammonia control.

48 Cisplatin-derived derivatives bearing phenylbutyrate and either crizotinib (complex 3) or ceri

49 inations of protein repair agents, such as 4-phenylbutyrate and genistein, may be necessary to restor

50 Sodium 4-phenylbutyrate and glycerol displayed a significant syne

51 cosahexaenoic acid in assembly disorders and phenylbutyrate and lovastatin in adrenoleukodystrophy (A

52 661) versus 977 (865) mumol.h/L for glycerol phenylbutyrate and NaPBA, respectively.

53 rtial-OTCD and 3 null-OTCD subjects received phenylbutyrate and phenylbutyrate plus BCAA supplementat

54 In contrast, sodium 4-phenylbutyrate and probenecid, the latter a uricosuric d

55 The chemical chaperones sodium 4-phenylbutyrate and tauroursodeoxycholic acid were found

56 s using small chemical chaperones, such as 4-phenylbutyrate and tauroursodeoxycholic acid, also atten

57 two different chemical chaperones, sodium 4-phenylbutyrate and tauroursodeoxycholic acid, both with

58 An oral sodium phenylbutyrate and taurursodiol combination (PB and TURS

59 Sodium phenylbutyrate and taurursodiol have been found to reduc

60 on were mimicked by HDAC inhibitors sodium 4-phenylbutyrate and trichostatin A (TSA).

61 h as pharmaceutical administration of sodium phenylbutyrate and/or ornithine and development of gene

62 ate, 3-(4-hydroxyphenyl)lactate, 2-hydroxy-4-phenylbutyrate, and 2-hydroxy-4-oxo-4-phenylbutyrate.

63 n expression in animal models, and butyrate, phenylbutyrate, and valproate induce gamma globin in hum

64 Treatment with SB and sodium phenylbutyrate, another HDACI, recovered cell viability

65 rols, including 27 degrees C rescue and 4 mm phenylbutyrate at 37 degrees C, were strongly positive.

66 These results show that administration of phenylbutyrate, at doses that are well tolerated in man,

67 of the central aromatic ring of a series of phenylbutyrate-based antagonists of the vitronectin rece

68 The ER stress inhibitor, 4-phenylbutyrate, blocked CITED2 knockdown-induced apoptos

69 Importantly, we found that sodium 4-phenylbutyrate (Buphenyl(R)), a drug used to treat urea

70 Sodium 4-phenylbutyrate (Buphenyl, 4PBA) is a new FDA approved dr

71 n of valproate, butyrate, phenylacetate, and phenylbutyrate by coupling them with Zn(2+)-chelating mo

72 d amino acid catabolism, which suggests that phenylbutyrate can be used to dispose of nitrogen effect

73 Furthermore, we show that 4-phenylbutyrate can rescue the enamel phenotype in affect

74 One hydroxamate-tethered phenylbutyrate compound, N-hydroxy-4-(4-phenylbutyrylami

75 e cells with the chemical chaperone sodium 4-phenylbutyrate could partially restore trafficking of mu

76 ino)benzamide (HTPB), a hydroxamate-tethered phenylbutyrate derivative with sub-micromolar potency in

77 at assessing the antitumor effect of a novel phenylbutyrate-derived histone deacetylase (HDAC) inhibi

78 cid HDAC inhibitors, such as isobutyrate and phenylbutyrate, did not reactivate EBV.

79 Treatment with the peroxisome proliferator 4-phenylbutyrate exerted protective effects with improved

80 Glycerol phenylbutyrate exhibits favorable pharmacokinetics and a

81 ction and characterization of metabolites of phenylbutyrate from serum samples of Huntington's diseas

82 Glycerol phenylbutyrate (GPB) lowers ammonia by providing an alte

83 Glycerol phenylbutyrate (GPB), a liquid triglyceride pro-drug of

84 Inactivation of client cargo sorting using 4-phenylbutyrate had opposing reciprocal effects on client

85 of VCP, MLN-273 (proteasome inhibitor), or 4-phenylbutyrate (histone deacetylase inhibitor).

86 ng a molecular explanation for the effect of phenylbutyrate in a subset of MSUD patients.

87 UC0-24hr was directionally lower on glycerol phenylbutyrate in each study, similar among all subgroup

88 with sodium phenylacetate/benzoate or sodium phenylbutyrate in urea cycle disorder patients has been

89 Administration of phenylbutyrate increased brain histone acetylation and d

90 rocytes with the chemical chaperone 4-sodium phenylbutyrate increased ERp29 expression, rescued Cx43

91 Phenylbutyrate increased mRNA for components of the ubiq

92 The chemical chaperon, 4-phenylbutyrate, increased the mutant solubility, reduced

93 found that the histone deacetylase inhibitor phenylbutyrate increases DJ-1 expression by 300% in the

94 In vivo phenylbutyrate increases the proportion of active hepati

95 and the transcriptional regulator, sodium 4-phenylbutyrate, inhibit the constitutively activated inw

96 Phenylbutyrate is a drug used in patients with urea cycl

97 Glycerol phenylbutyrate is under development for treatment of ure

98 ith the histone deacetylase inhibitor sodium phenylbutyrate, lung tumor development was significantly

99 t induce peroxisome proliferation, such as 4-phenylbutyrate, may have therapeutic potential in the tr

100 Further, lowering ER stress by 4-phenylbutyrate mitigated the enhanced immune stimulation

101 en the vesicles are lysed in the presence of phenylbutyrate, most of the Usher proteins cosediment in

102 Here, we delineate that sodium phenylbutyrate (NaPB), a Food and Drug Administration-ap

103 Here, we used sodium phenylbutyrate (NaPB), an accelerator of BCAA catabolism

104 Here we demonstrate that sodium phenylbutyrate (NaPB), an FDA-approved therapy for reduc

105 t with glycerol phenylbutyrate versus sodium phenylbutyrate (NaPBA) in adult UCD patients and the com

106 ent of urea cycle disorders (UCDs) as sodium phenylbutyrate (NaPBA), mediates waste nitrogen excretio

107 In vitro treatment with phenylbutyrate of control fibroblasts and lymphoblasts r

108 phenylbutyrate (administered as the pro-drug phenylbutyrate) (OP) combined are synergistic and produc

109 that administration of chemical chaperone 4-phenylbutyrate or genetic inhibition of ATF4 successfull

110 d, and intraperitoneal saline (placebo), OP, phenylbutyrate, or L-ornithine were administered after r

111 bination with sodium phenylacetate or sodium phenylbutyrate, or sodium phenylbutyrate alone.

112 Our study evaluated the combination of phenylbutyrate (PB) and 13-cis retinoic acid (CRA) as a

113 We previously reported that phenylbutyrate (PB), a differentiation agent, retarded t

114 ve patients were treated with repeat RA plus phenylbutyrate (PB), a histone deacetylase inhibitor, an

115 the well-known but less specific inhibitor, phenylbutyrate (PB), could partially reverse ETO-mediate

116 eding Drosophila throughout adulthood with 4-phenylbutyrate (PBA) can significantly increase lifespan

117 Sodium phenylbutyrate (PBA) is a derivative of the short-chain

118 nown to suppress ER stress signaling, like 4-phenylbutyrate (PBA) or tauroursodeoxycholic acid, will

119 marrow-derived dendritic cells (BMDCs) with phenylbutyrate (PBA) reduces lipopolysaccharide-induced

120 to determine whether topical ocular sodium 4-phenylbutyrate (PBA) treatment rescues glaucoma phenotyp

121 Systemic administration of sodium 4-phenylbutyrate (PBA), a chemical chaperone, increased pr

122 t with chemical chaperones, such as sodium 4-phenylbutyrate (PBA), reduces protein aggregation in neu

123 examine the role of three SCFA derivatives: phenylbutyrate (PBA), sodium butyrate (NaB), and sodium

124 aperones tauroursodeoxycholate (TUDCA) and 4-phenylbutyrate (PBA), which facilitate protein folding a

125 de proof of the concept that L-ornithine and phenylbutyrate/phenylacetate act synergistically to prod

126 ll-OTCD subjects received phenylbutyrate and phenylbutyrate plus BCAA supplementation.

127 Sodium phenylbutyrate preferentially reverses the cell death ca

128 duction of ER stress in the TM with sodium 4-phenylbutyrate prevented dexamethasone-induced ocular hy

129 Using recombinant enzymes, we show that phenylbutyrate prevents phosphorylation of E1alpha by in

130 In addition, glycerol and sodium 4-phenylbutyrate reduced the amount of Hsc70 expressed in

131 ically approved chemical chaperone, sodium 4-phenylbutyrate reduced the platelet hyperactivation.

132 wy body disease, long-term administration of phenylbutyrate reduces alpha-synuclein aggregation in br

133 Treatment with 4-phenylbutyrate resulted in remarkable amelioration of th

134 Colchicine and sodium 4-phenylbutyrate reverse these processes and could potenti

135 Phenylbutyrate showed promise in a mouse model and an op

136 ed UPR/ER stress by the chemical chaperone 4-phenylbutyrate significantly alleviated astrocyte-mediat

137 HDAC1 siRNA or other HDAC inhibitors (phenylbutyrate, sodium butyrate or trichostatin A) also

138 Oral sodium phenylbutyrate (SPB) is currently under investigation as

139 ent on IRE1b and to be inhibited by sodium 4-phenylbutyrate, suggesting that heat-induced autophagy i

140 ts raise the possibility that NaPA or sodium phenylbutyrate taken through drinking water or milk may

141 ly assigned in a 2:1 ratio to receive sodium phenylbutyrate-taurursodiol (3 g of sodium phenylbutyrat

142 137 were randomly assigned to receive sodium phenylbutyrate-taurursodiol (89 participants) or placebo

143 o evaluate the efficacy and safety of sodium phenylbutyrate-taurursodiol in persons with ALS.

144 Sodium phenylbutyrate-taurursodiol resulted in slower functiona

145 Coformulated sodium phenylbutyrate/taurursodiol (PB/TURSO) was shown to prol

146 CKA are both significantly reduced following phenylbutyrate therapy in control subjects and in patien

147 ible benefits of BCAA supplementation during phenylbutyrate therapy.

148 The administration of a test dose of sodium phenylbutyrate to the control group did not affect the r

149 aPBA in UCD patients, and long-term glycerol phenylbutyrate treatment in pediatric UCD patients was a

150 Colchicine and sodium 4-phenylbutyrate treatment increased secretion of THP from

151 In mice, phenylbutyrate treatment leads to a 260% increase in bra

152 Phenylbutyrate treatment may be a valuable treatment for

153 We show that 4-phenylbutyrate treatment of cells from both X-ALD patien

154 dies involving short- and long-term glycerol phenylbutyrate treatment of UCD patients ages 6 and abov

155 emonstrate the in vivo efficacy of dietary 4-phenylbutyrate treatment through its production of a sub

156 bservation, we investigated the potential of phenylbutyrate treatment to lower BCAA and their corresp

157 ion in plasma concentrations of BCAAs due to phenylbutyrate treatment was observed.

158 After 4-phenylbutyrate treatment, an increase in transcription o

159 During 12 months of open-label glycerol phenylbutyrate treatment, average ammonia was normal in

160 age and with sodium phenylacetate or sodium phenylbutyrate treatment.

161 Low protein diet and sodium or glycerol phenylbutyrate, two pillars of recommended long-term the

162 d by treatment with the chemical chaperone 4-phenylbutyrate, uncovering the involvement of ER stress

163 designed short-term comparisons of glycerol phenylbutyrate versus NaPBA, NH3 -AUC0-24hr was directio

164 rmacokinetics during treatment with glycerol phenylbutyrate versus sodium phenylbutyrate (NaPBA) in a

165 m rapamycin, 1000 ppm acarbose, and 1000 ppm phenylbutyrate was fed to 20-month-old C57BL/6 and HET3

166 Glycerol phenylbutyrate was noninferior to NaPBA with respect to

167 onoscavenger therapy with sodium or glycerol phenylbutyrate was superior to sodium benzoate in provid

168 aperones (thapsigargin, glycerol or sodium 4-phenylbutyrate), we demonstrated a marked increase in ce

169 Individually, L-ornithine or phenylbutyrate were similar to the BDL group.