ライフサイエンスコーパス: phenylbutyrate

1 ll SCFAs that are HDAC inhibitors, including phenylbutyrate.

2 administration of the molecular chaperone 4-phenylbutyrate.

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

4 by the histone deacetylase inhibitor sodium phenylbutyrate.

5 of the histone deacetylase inhibitor sodium phenylbutyrate.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

26 However, phenylbutyrate administration decreases plasma branched-

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

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

29 Prolonged phenylbutyrate administration reduced ureagenesis and th

30 Phenylbutyrate administration reduced ureagenesis by app

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

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

33 lacetate or sodium phenylbutyrate, or sodium phenylbutyrate alone.

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

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

36 Sodium 4-phenylbutyrate and glycerol displayed a significant syne

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

60 Glycerol phenylbutyrate exhibits favorable pharmacokinetics and a

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

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

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

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

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

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

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

68 Administration of phenylbutyrate increased brain histone acetylation and d

69 Phenylbutyrate increased mRNA for components of the ubiq

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

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

72 In vivo phenylbutyrate increases the proportion of active hepati

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

74 Phenylbutyrate is a drug used in patients with urea cycl

75 Glycerol phenylbutyrate is under development for treatment of ure

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

108 Phenylbutyrate showed promise in a mouse model and an op

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

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

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

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

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

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

115 ible benefits of BCAA supplementation during phenylbutyrate therapy.

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

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

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

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

120 Phenylbutyrate treatment may be a valuable treatment for

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

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

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

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

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

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

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

128 age and with sodium phenylacetate or sodium phenylbutyrate treatment.

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

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

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

132 Glycerol phenylbutyrate was noninferior to NaPBA with respect to

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

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