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2020 3
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Progressive familial intrahepatic cholestasis.
Srivastava A. Srivastava A. J Clin Exp Hepatol. 2014 Mar;4(1):25-36. doi: 10.1016/j.jceh.2013.10.005. Epub 2013 Nov 23. J Clin Exp Hepatol. 2014. PMID: 25755532 Free PMC article. Review.
The main clinical presentation is in the form of cholestatic jaundice and pruritus. Serum gamma glutamyl transpeptidase (GGT) is normal in patients with PFIC1/2 while it is raised in patients with PFIC3. ...
The main clinical presentation is in the form of cholestatic jaundice and pruritus. Serum gamma glutamyl transpeptidase (GGT) is norm …
High-fat diet modifies the PPAR-gamma pathway leading to disruption of microbial and physiological ecosystem in murine small intestine.
Tomas J, Mulet C, Saffarian A, Cavin JB, Ducroc R, Regnault B, Kun Tan C, Duszka K, Burcelin R, Wahli W, Sansonetti PJ, Pédron T. Tomas J, et al. Proc Natl Acad Sci U S A. 2016 Oct 4;113(40):E5934-E5943. doi: 10.1073/pnas.1612559113. Epub 2016 Sep 16. Proc Natl Acad Sci U S A. 2016. PMID: 27638207 Free PMC article.
In addition, HF diet increased intestinal permeability and decreased cystic fibrosis transmembrane conductance regulator (Cftr) and the Na-K-2Cl cotransporter 1 (Nkcc1) gene and protein expressions, leading to a decrease in ileal secretion of chloride, likely responsible f …
In addition, HF diet increased intestinal permeability and decreased cystic fibrosis transmembrane conductance regulator (Cftr) and t …
Ineffective correction of PPARgamma signaling in cystic fibrosis airway epithelial cells undergoing repair.
Bou Saab J, Bacchetta M, Chanson M. Bou Saab J, et al. Int J Biochem Cell Biol. 2016 Sep;78:361-369. doi: 10.1016/j.biocel.2016.07.035. Epub 2016 Jul 30. Int J Biochem Cell Biol. 2016. PMID: 27484450
Peroxisome proliferator-activated receptor gamma (PPARgamma) represents a potential target to treat airway mucus hypersecretion in cystic fibrosis (CF). ...These changes were accompanied by an increase in the expression of MUC5B. The correlation between PPARgamma
Peroxisome proliferator-activated receptor gamma (PPARgamma) represents a potential target to treat airway mucus hypersecretio …
PPARgamma as a therapeutic target in cystic fibrosis.
Dekkers JF, van der Ent CK, Kalkhoven E, Beekman JM. Dekkers JF, et al. Trends Mol Med. 2012 May;18(5):283-91. doi: 10.1016/j.molmed.2012.03.004. Epub 2012 Apr 9. Trends Mol Med. 2012. PMID: 22494945 Review.
Cystic fibrosis (CF) is characterized by a proinflammatory pulmonary condition that may result from increased infections and altered intracellular metabolism in CFTR-deficient cells. The lipid-activated transcription factor peroxisome proliferator-activated receptor-gam
Cystic fibrosis (CF) is characterized by a proinflammatory pulmonary condition that may result from increased infections and altered intrace …
Inhibition of Sphingolipid Synthesis as a Phenotype-Modifying Therapy in Cystic Fibrosis.
Mingione A, Dei Cas M, Bonezzi F, Caretti A, Piccoli M, Anastasia L, Ghidoni R, Paroni R, Signorelli P. Mingione A, et al. Cell Physiol Biochem. 2020 Jan 31;54(1):110-125. doi: 10.33594/000000208. Cell Physiol Biochem. 2020. PMID: 31999897 Free article.
BACKGROUND/AIMS: Cystic Fibrosis (CF) is an inherited disease associated with a variety of mutations affecting the CFTR gene. A deletion of phenylalanine 508 (F508) affects more than 70% of patients and results in unfolded proteins accumulation, originating a proteinopathy …
BACKGROUND/AIMS: Cystic Fibrosis (CF) is an inherited disease associated with a variety of mutations affecting the CFTR gene. A delet …
PPAR-gamma agonists in polycystic kidney disease with frequent development of cardiovascular disorders.
Nagao S, Yamaguchi T. Nagao S, et al. Curr Mol Pharmacol. 2012 Jun;5(2):292-300. doi: 10.2174/1874467211205020292. Curr Mol Pharmacol. 2012. PMID: 22122459 Review.
PPAR-gamma agonists ameliorate polycystic kidney, polycystic liver and cardiac defects through beta-catenin, c-Myc, CFTR, MCP-1, S6, ERK, and TGF-beta signaling pathways in animal models of PKD. In this review, we describe the possible therapeutic value of
PPAR-gamma agonists ameliorate polycystic kidney, polycystic liver and cardiac defects through beta-catenin, c-Myc, CFTR
UPR modulation of host immunity by Pseudomonas aeruginosa in cystic fibrosis.
Bedi B, Lin KC, Maurice NM, Yuan Z, Bijli K, Koval M, Hart CM, Goldberg JB, Stecenko A, Sadikot RT. Bedi B, et al. Clin Sci (Lond). 2020 Jul 31;134(14):1911-1934. doi: 10.1042/CS20200066. Clin Sci (Lond). 2020. PMID: 32537652
The nuclear hormone receptor peroxisome proliferator-activated receptor (PPAR) gamma (PPARgamma), has shown to be reduced in CF airways. In the present study, we sought to investigate the upstream mechanisms repressing PPARgamma expression and its impa …
The nuclear hormone receptor peroxisome proliferator-activated receptor (PPAR) gamma (PPARgamma), has shown to be reduc …
Stimulation of nuclear receptor peroxisome proliferator-activated receptor-gamma limits NF-kappaB-dependent inflammation in mouse cystic fibrosis biliary epithelium.
Scirpo R, Fiorotto R, Villani A, Amenduni M, Spirli C, Strazzabosco M. Scirpo R, et al. Hepatology. 2015 Nov;62(5):1551-62. doi: 10.1002/hep.28000. Epub 2015 Aug 28. Hepatology. 2015. PMID: 26199136 Free PMC article.
Stimulation of PPAR-gamma in vivo (rosiglitazone) significantly attenuates biliary damage and inflammation in Cftr-knockout mice exposed to a dextran sodium sulfate-induced portal endotoxemia. CONCLUSION: These studies unravel a novel function of PPAR- …
Stimulation of PPAR-gamma in vivo (rosiglitazone) significantly attenuates biliary damage and inflammation in Cftr-knoc …
Fenretinide differentially modulates the levels of long- and very long-chain ceramides by downregulating Cers5 enzyme: evidence from bench to bedside.
Garić D, De Sanctis JB, Wojewodka G, Houle D, Cupri S, Abu-Arish A, Hanrahan JW, Hajduch M, Matouk E, Radzioch D. Garić D, et al. J Mol Med (Berl). 2017 Oct;95(10):1053-1064. doi: 10.1007/s00109-017-1564-y. Epub 2017 Jul 10. J Mol Med (Berl). 2017. PMID: 28695226 Clinical Trial.
Our results show that restoration of cystic fibrosis conductance regulator (CFTR) expression is associated with normalization of aberrant levels of specific ceramides. This demonstrates for the first time a correlation between CFTR protein expression and regulation …
Our results show that restoration of cystic fibrosis conductance regulator (CFTR) expression is associated with normalization of aber …
Cystic fibrosis: a disorder with defective autophagy.
Luciani A, Villella VR, Esposito S, Brunetti-Pierri N, Medina DL, Settembre C, Gavina M, Raia V, Ballabio A, Maiuri L. Luciani A, et al. Autophagy. 2011 Jan;7(1):104-6. doi: 10.4161/auto.7.1.13987. Epub 2011 Jan 1. Autophagy. 2011. PMID: 21048426
A sequestration of misfolded cystic: fibrosis transmembrane conductance regulator (CFTR) and cross-linked PPARgamma has been observed in airway epithelia of cystic fibrosis (CF) patients. ...Therefore, rescuing autophagy interrupts the vicious cycle linking defectiv …
A sequestration of misfolded cystic: fibrosis transmembrane conductance regulator (CFTR) and cross-linked PPARgamma has been o …
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