[1] Banales JM, Huebert RC, Karlsen T, et al. Cholangiocyte pathobiology[J]. Nat Rev Gastroenterol Hepatol, 2019, 16(5): 269-281.
[2] Dawson PA, Haywood J, Craddock AL, et al. Targeted deletion of the ileal bile acid transporter eliminates enterohepatic cycling of bile acids in mice[J]. J Biol Chem, 2003, 278(36): 33920-33927.
[3] Van Wessel DBE, Thompson RJ, Gonzales E, et al. Impact of Genotype, Serum Bile Acids, and Surgical Biliary Diversion on Native Liver Survival in FIC1 Deficiency[J]. Hepatology, 2021, 74(2): 892-906.
[4] Nevens F, Trauner M, Manns MP. Primary biliary cholangitis as a roadmap for the development of novel treatments for cholestatic liver diseases?[J]. J Hepatol, 2023, 78(2): 430-441.
[5] Tian S, Chen S, Pan C, et al. FXR: structures, biology, and drug development for NASH and fibrosis diseases[J]. Acta Pharmacol Sin, 2022, 43(5): 1120-1132.
[6] Beuers U. New paradigms in the treatment of hepatic cholestasis: From UDCA to FXR, PXR and beyond[J]. J Hepatol, 2015, 62(1 Suppl):S25-37.
[7] Halilbasic E, Claudel T, Trauner M. Bile acid transporters and regulatory nuclear receptors in the liver and beyond[J]. J Hepatol, 2013, 58(1): 155-168.
[8] Trauner M, Fuchs CD. Novel therapeutic targets for cholestatic and fatty liver disease[J]. Gut, 2022, 71(1): 194-209.
[9] John BV, Schwartz K, Levy C, et al. Impact of Obeticholic acid Exposure on Decompensation and Mortality in Primary Biliary Cholangitis and Cirrhosis[J]. Hepatol Commun, 2021, 5(8)5(8):1426-1436.
[10] Fiorucci S,Distrutti E. Bile acids and their receptors[M]. Cham, Switzerland: Springer, 2019.
[11] Trauner M, Gulamhusein A, Hameed B, et al. The Nonsteroidal farnesoid X receptor agonist Cilofexor (GS‐9674) improves markers of cholestasis and liver injury in patients with primary sclerosing cholangitis[J]. Hepatology, 2019, 70(3): 788-801.
[12] Gadaleta RM, Scialpi N, Peres C, et al. Suppression of Hepatic Bile Acid Synthesis by a non-tumorigenic FGF19 analogue Protects Mice from Fibrosis and Hepatocarcinogenesis[J]. Sci Rep, 2018, 8(1): 17210.
[13] Harrison SA, Rinella ME, Abdelmalek MF, et al. NGM282 for treatment of non-alcoholic steatohepatitis: a multicentre, randomised, double-blind, placebo-controlled, phase 2 trial[J]. Lancet, 2018, 391(10126): 1174-1185.
[14] Ghonem NS, Assis DN, Boyer JL. On fibrates and cholestasis: A review[J]. 2015.
[15] Corpechot C, Chazouillères O, Rousseau A, et al. A Placebo-Controlled Trial of Bezafibrate in Primary Biliary Cholangitis[J]. N Engl J Med, 2018, 378(23): 2171-2181.
[16] Jones D, Boudes PF, Swain MG, et al. Seladelpar (MBX-8025), a selective PPAR-δ agonist, in patients with primary biliary cholangitis with an inadequate response to ursodeoxycholic acid: a double-blind, randomised, placebo-controlled, phase 2, proof-of-concept study[J]. Lancet Gastroenterol Hepatol, 2017, 2(10): 716-726.
[17] Thompson RJ, Arnell H, Artan R, et al. Odevixibat treatment in progressive familial intrahepatic cholestasis: a randomised, placebo-controlled, phase 3 trial[J]. Lancet Gastroenterol Hepatol, 2022, 7(9): 830-842.
[18] Gonzales E, Hardikar W, Stormon M, et al. Efficacy and safety of maralixibat treatment in patients with Alagille syndrome and cholestatic pruritus (ICONIC): a randomised phase 2 study[J]. Lancet, 2021, 398(10311): 1581-1592.
[19] Miethke AG, Zhang W, Simmons J, et al. Pharmacological inhibition of apical sodium-dependent bile acid transporter changes bile composition and blocks progression of sclerosing cholangitis in multidrug resistance 2 knockout mice[J]. Hepatology (Baltimore, Md.), 2016, 63(2): 512-523.
[20] Baghdasaryan A, Fuchs CD, Österreicher CH, et al. Inhibition of intestinal bile acid absorption improves cholestatic liver and bile duct injury in a mouse model of sclerosing cholangitis[J]. J Hepatol, 2016, 64(3): 674-681.
[21] Hegade VS, Kendrick SFW, Dobbins RL, et al. Effect of ileal bile acid transporter inhibitor GSK2330672 on pruritus in primary biliary cholangitis: a double-blind, randomised, placebo-controlled, crossover, phase 2a study[J]. Lancet (London, England), 2017, 389(10074): 1114-1123.
[22] Mayo MJ, Pockros PJ, Jones D, et al. A randomized, controlled, phase 2 study of Maralixibat in the treatment of itching associated with primary biliary cholangitis[J]. Hepatol Commun, 2019, 3(3): 365-381.
[23] Levy C, Kendrick S, Bowlus CL, et al. GLIMMER: A randomized phase 2b dose-ranging trial of Linerixibat in primary biliary cholangitis patients with pruritus[J]. Clin Gastroenterol Hepatol, 2023, 21(7): 1902-1912.e13.
[24] Caballero-Camino FJ, Rodrigues PM, Wångsell F, et al. A3907, a systemic ASBT inhibitor, improves cholestasis in mice by multiorgan activity and shows translational relevance to humans[J]. Hepatology, 2023, 78(3): 709-726.
[25] Sauter G, Berr F, Beuers U, et al. Serum concentrations of 7alpha-hydroxy-4-cholesten-3-one reflect bile acid synthesis in humans[J]. Hepatology, 1996, 24(1): 123-126. |
