PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 10720936-4 2000 The hypothesis of this discussion is that the short chain fatty acids, butyrate and 4-phenylbutyrate, up-regulate mature CFTR at the plasma membrane. 4-phenylbutyric acid 84-100 CF transmembrane conductance regulator Homo sapiens 121-125 10666019-0 2000 Focus on "Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR". 4-phenylbutyric acid 10-33 CF transmembrane conductance regulator Homo sapiens 111-115 10666020-0 2000 Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR. 4-phenylbutyric acid 0-23 CF transmembrane conductance regulator Homo sapiens 101-105 10666020-2 2000 Sodium 4-phenylbutyrate (4PBA) improves DeltaF508-CFTR trafficking and function in vitro in cystic fibrosis epithelial cells and in vivo. 4-phenylbutyric acid 0-23 CF transmembrane conductance regulator Homo sapiens 50-54 33038073-4 2020 CFTR activity in homozygous F508del CFTR epithelia was rescued by treatment with VX-661, C4/C18, 4-phenylbutyrate (4-PBA) for 24 hr at 37 C or by incubation at 29 C for 48 hr. 4-phenylbutyric acid 97-113 CF transmembrane conductance regulator Homo sapiens 0-4 9476862-0 1998 A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function. 4-phenylbutyric acid 31-54 CF transmembrane conductance regulator Homo sapiens 156-160 9366560-0 1997 In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. 4-phenylbutyric acid 76-99 CF transmembrane conductance regulator Homo sapiens 38-42 9366560-0 1997 In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. 4-phenylbutyric acid 76-99 CF transmembrane conductance regulator Homo sapiens 158-162 9366560-2 1997 We hypothesize that a known transcriptional regulator, sodium 4-phenylbutyrate (4PBA), will enable a greater fraction of delta F508-CFTR to escape degradation and appear at the cell surface. 4-phenylbutyric acid 55-78 CF transmembrane conductance regulator Homo sapiens 132-136 33038073-4 2020 CFTR activity in homozygous F508del CFTR epithelia was rescued by treatment with VX-661, C4/C18, 4-phenylbutyrate (4-PBA) for 24 hr at 37 C or by incubation at 29 C for 48 hr. 4-phenylbutyric acid 97-113 CF transmembrane conductance regulator Homo sapiens 36-40 33038073-4 2020 CFTR activity in homozygous F508del CFTR epithelia was rescued by treatment with VX-661, C4/C18, 4-phenylbutyrate (4-PBA) for 24 hr at 37 C or by incubation at 29 C for 48 hr. 4-phenylbutyric acid 115-120 CF transmembrane conductance regulator Homo sapiens 0-4 33038073-4 2020 CFTR activity in homozygous F508del CFTR epithelia was rescued by treatment with VX-661, C4/C18, 4-phenylbutyrate (4-PBA) for 24 hr at 37 C or by incubation at 29 C for 48 hr. 4-phenylbutyric acid 115-120 CF transmembrane conductance regulator Homo sapiens 36-40 26368820-8 2015 4-Phenylbutyrate has been used to stabilize DeltaPhe508 mutant cystic fibrosis transmembrane conductance regulator protein and is in clinical use in patients with urea cycle defects. 4-phenylbutyric acid 0-16 CF transmembrane conductance regulator Homo sapiens 63-114 16472777-2 2006 4-Phenylbutyrate corrects defective DeltaF508-CFTR trafficking in vitro, but is not clinically efficacious. 4-phenylbutyric acid 0-16 CF transmembrane conductance regulator Homo sapiens 46-50 26115671-13 2015 4-PBA-treated CuFi-5 cells also exhibited an increase in forskolin-induced CFTR-mediated currents. 4-phenylbutyric acid 0-5 CF transmembrane conductance regulator Homo sapiens 75-79 22069317-1 2011 Sodium 4-phenylbutyrate (4PBA) corrects trafficking of DeltaF508-CFTR in Cystic Fibrosis (CF) epithelia, which is hypothesized to, at least in part, result from increased expression of Hsp70 (stress-induced 70 kDa heat shock protein). 4-phenylbutyric acid 0-23 CF transmembrane conductance regulator Homo sapiens 65-69 20935229-9 2011 Incubation with 1 mM 4-phenylbutyrate synergistically increased Dex-induced ENaC functional expression in DeltaF508-CFTR-overexpressing cells. 4-phenylbutyric acid 21-37 CF transmembrane conductance regulator Homo sapiens 116-120 18285607-9 2008 A subset of 4-PBA-modulated ER-associated degradation chaperones (GRP94, HSP84, GRP78, GRP75, and GRP58) was observed to associate with the immature B form of CFTR in ER. 4-phenylbutyric acid 12-17 CF transmembrane conductance regulator Homo sapiens 159-163 17890229-1 2007 Sodium 4-phenylbutyrate (4-PBA) has been shown to correct the cellular trafficking of several mutant or nonmutant plasma membrane proteins such as cystic fibrosis transmembrane conductance regulator through the expression of 70-kDa heat shock proteins. 4-phenylbutyric acid 0-23 CF transmembrane conductance regulator Homo sapiens 147-198 17890229-1 2007 Sodium 4-phenylbutyrate (4-PBA) has been shown to correct the cellular trafficking of several mutant or nonmutant plasma membrane proteins such as cystic fibrosis transmembrane conductance regulator through the expression of 70-kDa heat shock proteins. 4-phenylbutyric acid 25-30 CF transmembrane conductance regulator Homo sapiens 147-198 16738016-6 2006 Last, the activity of the HKalpha2 S955/A was rescued by treatment with 4-phenylbutyric acid, a compound that was shown previously to restore function to the cystic fibrosis transmembrane conductance regulator. 4-phenylbutyric acid 72-92 CF transmembrane conductance regulator Homo sapiens 158-209 21525008-1 2011 Sodium 4-phenylbutyrate (4PBA) improves the intracellular trafficking of DeltaF508-CFTR in cystic fibrosis (CF) epithelial cells. 4-phenylbutyric acid 0-23 CF transmembrane conductance regulator Homo sapiens 83-87 18574003-0 2008 Proinflammatory effect of sodium 4-phenylbutyrate in deltaF508-cystic fibrosis transmembrane conductance regulator lung epithelial cells: involvement of extracellular signal-regulated protein kinase 1/2 and c-Jun-NH2-terminal kinase signaling. 4-phenylbutyric acid 26-49 CF transmembrane conductance regulator Homo sapiens 63-114 18574003-1 2008 Sodium 4-phenylbutyrate (4-PBA) has attracted a great deal of attention in cystic fibrosis (CF) pathology due to its capacity to traffic DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) to the cell membrane and restore CFTR chloride function at the plasma membrane of CF lung cells in vitro and in vivo. 4-phenylbutyric acid 0-23 CF transmembrane conductance regulator Homo sapiens 146-198 18574003-1 2008 Sodium 4-phenylbutyrate (4-PBA) has attracted a great deal of attention in cystic fibrosis (CF) pathology due to its capacity to traffic DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) to the cell membrane and restore CFTR chloride function at the plasma membrane of CF lung cells in vitro and in vivo. 4-phenylbutyric acid 0-23 CF transmembrane conductance regulator Homo sapiens 200-204 18574003-1 2008 Sodium 4-phenylbutyrate (4-PBA) has attracted a great deal of attention in cystic fibrosis (CF) pathology due to its capacity to traffic DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) to the cell membrane and restore CFTR chloride function at the plasma membrane of CF lung cells in vitro and in vivo. 4-phenylbutyric acid 0-23 CF transmembrane conductance regulator Homo sapiens 239-243 18574003-1 2008 Sodium 4-phenylbutyrate (4-PBA) has attracted a great deal of attention in cystic fibrosis (CF) pathology due to its capacity to traffic DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) to the cell membrane and restore CFTR chloride function at the plasma membrane of CF lung cells in vitro and in vivo. 4-phenylbutyric acid 25-30 CF transmembrane conductance regulator Homo sapiens 146-198 18574003-1 2008 Sodium 4-phenylbutyrate (4-PBA) has attracted a great deal of attention in cystic fibrosis (CF) pathology due to its capacity to traffic DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) to the cell membrane and restore CFTR chloride function at the plasma membrane of CF lung cells in vitro and in vivo. 4-phenylbutyric acid 25-30 CF transmembrane conductance regulator Homo sapiens 200-204 18574003-1 2008 Sodium 4-phenylbutyrate (4-PBA) has attracted a great deal of attention in cystic fibrosis (CF) pathology due to its capacity to traffic DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) to the cell membrane and restore CFTR chloride function at the plasma membrane of CF lung cells in vitro and in vivo. 4-phenylbutyric acid 25-30 CF transmembrane conductance regulator Homo sapiens 239-243 18574003-3 2008 This 4-PBA-induced IL-8 production was associated with a strong reduction of proteasome and nuclear factor-kappaB transcriptional activities in the two DeltaF508-CFTR lung cells either in a resting state or after tumor necrosis factor-alpha stimulation. 4-phenylbutyric acid 5-10 CF transmembrane conductance regulator Homo sapiens 162-166 18574003-7 2008 Therefore, we suggest that inhibition of both ERK1/2 and JNK signaling may be a means to strongly reduce 4-PBA-induced IL-8 production in combination with 4-PBA treatment to restore CFTR Cl(-) channel function in lung epithelial cells of patients with CF. 4-phenylbutyric acid 105-110 CF transmembrane conductance regulator Homo sapiens 182-186 18574003-7 2008 Therefore, we suggest that inhibition of both ERK1/2 and JNK signaling may be a means to strongly reduce 4-PBA-induced IL-8 production in combination with 4-PBA treatment to restore CFTR Cl(-) channel function in lung epithelial cells of patients with CF. 4-phenylbutyric acid 155-160 CF transmembrane conductance regulator Homo sapiens 182-186 12095312-0 2002 Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate. 4-phenylbutyric acid 78-94 CF transmembrane conductance regulator Homo sapiens 12-16 10853862-0 2000 Activation of deltaF508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX. 4-phenylbutyric acid 86-102 CF transmembrane conductance regulator Homo sapiens 24-28 11404244-1 2001 Intracellular trafficking of the DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) is repaired by sodium 4-phenylbutyrate (4PBA) by an undetermined mechanism. 4-phenylbutyric acid 117-140 CF transmembrane conductance regulator Homo sapiens 96-100 11404246-4 2001 IB3-1 cells were treated with sodium 4-phenylbutyrate (4PBA) to promote maturation of DeltaF508 CFTR to band C. A dose-dependent increase in band C and total cellular HSP70 was observed. 4-phenylbutyric acid 30-53 CF transmembrane conductance regulator Homo sapiens 96-100 11164382-2 2001 Here it is shown, using patch clamp recording from CFTR-transfected mammalian cell lines, that the fatty acids butyrate and 4-phenylbutyrate cause a voltage-dependent block of CFTR Cl(-) currents when applied to the cytoplasmic face of membrane patches, with apparent K(d)s (at 0 mV) of 29.6 mM for butyrate and 6.6 mM for 4-phenylbutyrate. 4-phenylbutyric acid 124-140 CF transmembrane conductance regulator Homo sapiens 51-55 11164382-2 2001 Here it is shown, using patch clamp recording from CFTR-transfected mammalian cell lines, that the fatty acids butyrate and 4-phenylbutyrate cause a voltage-dependent block of CFTR Cl(-) currents when applied to the cytoplasmic face of membrane patches, with apparent K(d)s (at 0 mV) of 29.6 mM for butyrate and 6.6 mM for 4-phenylbutyrate. 4-phenylbutyric acid 124-140 CF transmembrane conductance regulator Homo sapiens 176-180 11164382-2 2001 Here it is shown, using patch clamp recording from CFTR-transfected mammalian cell lines, that the fatty acids butyrate and 4-phenylbutyrate cause a voltage-dependent block of CFTR Cl(-) currents when applied to the cytoplasmic face of membrane patches, with apparent K(d)s (at 0 mV) of 29.6 mM for butyrate and 6.6 mM for 4-phenylbutyrate. 4-phenylbutyric acid 323-339 CF transmembrane conductance regulator Homo sapiens 176-180 11164382-4 2001 The concentration-dependence of block suggests that CFTR-mediated Cl(-) currents in vivo may be affected by both 4-phenylbutyrate used in the treatment of various diseases, including cystic fibrosis, and by butyrate produced endogenously within the colonic lumen. 4-phenylbutyric acid 113-129 CF transmembrane conductance regulator Homo sapiens 52-56 10853862-8 2000 The increased efflux of chloride after 4-phenylbutyrate treatment can be explained by the fact that 4-phenylbutyrate allows the deltaF508 cystic fibrosis transmembrane conductance regulator to escape degradation and to be transported to the cell surface. 4-phenylbutyric acid 39-55 CF transmembrane conductance regulator Homo sapiens 138-189 10853862-8 2000 The increased efflux of chloride after 4-phenylbutyrate treatment can be explained by the fact that 4-phenylbutyrate allows the deltaF508 cystic fibrosis transmembrane conductance regulator to escape degradation and to be transported to the cell surface. 4-phenylbutyric acid 100-116 CF transmembrane conductance regulator Homo sapiens 138-189