Pages that link to "Q34136693"
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The following pages link to Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate (Q34136693):
Displaying 50 items.
- Molecular Chaperones as Targets to Circumvent the CFTR Defect in Cystic Fibrosis (Q21129282) (← links)
- Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (Q24187139) (← links)
- Disease-associated mutations affect intracellular traffic and paracellular Mg2+ transport function of Claudin-16 (Q24544268) (← links)
- Chloride channels as drug targets (Q24601830) (← links)
- Clinical and experimental applications of sodium phenylbutyrate (Q24613511) (← links)
- Acute kidney injury: preclinical innovations, challenges, and opportunities for translation (Q26786667) (← links)
- Progress towards genetic and pharmacological therapies for keratin genodermatoses: current perspective and future promise (Q28393706) (← links)
- A trafficking-deficient mutant of KCC3 reveals dominant-negative effects on K-Cl cotransport function (Q28486016) (← links)
- Abnormal trafficking of endogenously expressed BMPR2 mutant allelic products in patients with heritable pulmonary arterial hypertension (Q28534831) (← links)
- Restoration of mutant bestrophin-1 expression, localisation and function in a polarised epithelial cell model (Q28820977) (← links)
- Augmentation of CFTR maturation by S-nitrosoglutathione reductase (Q30277961) (← links)
- S-Nitrosothiols increases cystic fibrosis transmembrane regulator expression and maturation in the cell surface (Q30408475) (← links)
- Concentration-dependent effects of endogenous S-nitrosoglutathione on gene regulation by specificity proteins Sp3 and Sp1. (Q30432918) (← links)
- Hsp 70/Hsp 90 organizing protein as a nitrosylation target in cystic fibrosis therapy (Q30433825) (← links)
- Additive effect of multiple pharmacological chaperones on maturation of CFTR processing mutants (Q33286071) (← links)
- ER stress dependent microparticles derived from smooth muscle cells promote endothelial dysfunction during thoracic aortic aneurysm and dissection (Q33772309) (← links)
- Basic science of pulmonary arterial hypertension for clinicians: new concepts and experimental therapies (Q33849207) (← links)
- CFTR Modulators for the Treatment of Cystic Fibrosis (Q33920366) (← links)
- Elevated phenylacetic acid levels do not correlate with adverse events in patients with urea cycle disorders or hepatic encephalopathy and can be predicted based on the plasma PAA to PAGN ratio. (Q33939797) (← links)
- Regulation of endogenous ENaC functional expression by CFTR and ΔF508-CFTR in airway epithelial cells. (Q34502195) (← links)
- Phenylbutyrate therapy for maple syrup urine disease (Q34505609) (← links)
- Surface expression of ASIC2 inhibits the amiloride-sensitive current and migration of glioma cells (Q34527019) (← links)
- A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial (Q34662809) (← links)
- Phenyl acyl acids attenuate the unfolded protein response in tunicamycin-treated neuroblastoma cells (Q34970563) (← links)
- Therapeutic approaches to repair defects in deltaF508 CFTR folding and cellular targeting. (Q35012417) (← links)
- ERp29 regulates DeltaF508 and wild-type cystic fibrosis transmembrane conductance regulator (CFTR) trafficking to the plasma membrane in cystic fibrosis (CF) and non-CF epithelial cells (Q35065145) (← links)
- Chemical chaperones--a new concept in drug research (Q35097487) (← links)
- Pharmacological approaches to correcting the ion transport defect in cystic fibrosis (Q35627639) (← links)
- 4-Phenylbutyrate stimulates Hsp70 expression through the Elp2 component of elongator and STAT-3 in cystic fibrosis epithelial cells (Q35639867) (← links)
- Lung inflammation as a therapeutic target in cystic fibrosis. (Q35894285) (← links)
- A cure for traffic jams: small molecule chaperones in the endoplasmic reticulum (Q35915766) (← links)
- Low temperature and chemical rescue affect molecular proximity of DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) and epithelial sodium channel (ENaC) (Q35956664) (← links)
- Strategic targets of essential host-pathogen interactions (Q36064617) (← links)
- Cystic fibrosis lung disease: genetic influences, microbial interactions, and radiological assessment (Q36114460) (← links)
- Targeted therapy for cystic fibrosis: cystic fibrosis transmembrane conductance regulator mutation-specific pharmacologic strategies (Q36615230) (← links)
- CFTR: folding, misfolding and correcting the ΔF508 conformational defect. (Q36814102) (← links)
- N-glycan structure dictates extension of protein folding or onset of disposal (Q36825595) (← links)
- Airway epithelial control of Pseudomonas aeruginosa infection in cystic fibrosis. (Q36968815) (← links)
- Cystic fibrosis and estrogens: a perfect storm (Q36972780) (← links)
- Targeting histone deacetylases for the treatment of disease (Q37296159) (← links)
- Targeting HSP90 and monoclonal protein trafficking modulates the unfolded protein response, chaperone regulation and apoptosis in myeloma cells (Q37423748) (← links)
- Asbestos-induced alveolar epithelial cell apoptosis. The role of endoplasmic reticulum stress response. (Q37596494) (← links)
- Pharmacological therapy for cystic fibrosis: from bench to bedside (Q37887169) (← links)
- Targeting Huntington's disease through histone deacetylases (Q38019159) (← links)
- Cystic fibrosis transmembrane regulator correctors and potentiators (Q38118742) (← links)
- The therapeutic potential of chemical chaperones in protein folding diseases. (Q38211124) (← links)
- BMP type II receptor as a therapeutic target in pulmonary arterial hypertension. (Q38817806) (← links)
- Phenylbutyrate-a pan-HDAC inhibitor-suppresses proliferation of glioblastoma LN-229 cell line. (Q38844777) (← links)
- Cystic fibrosis: a clinical view (Q38973292) (← links)
- An update on the use of benzoate, phenylacetate and phenylbutyrate ammonia scavengers for interrogating and modifying liver nitrogen metabolism and its implications in urea cycle disorders and liver disease (Q39012545) (← links)