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1
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47749136267
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IKK1 and IKK2 cooperate to maintain bile duct integrity in the liver
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Combined loss of two important intracellular kinases led to the spontaneous development of jaundice and fatal cholangitis in mice. This process was suggested to be secondary to changes in the regulation of tight junctions in biliary epithelial cells
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Luedde T, Heinrichsdorff J, de Lorenzi R, et al. IKK1 and IKK2 cooperate to maintain bile duct integrity in the liver. Proc Natl Acad Sci U S A 2008; 105:9733-9738. Combined loss of two important intracellular kinases led to the spontaneous development of jaundice and fatal cholangitis in mice. This process was suggested to be secondary to changes in the regulation of tight junctions in biliary epithelial cells.
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(2008)
Proc Natl Acad Sci U S A
, vol.105
, pp. 9733-9738
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Luedde, T.1
Heinrichsdorff, J.2
De Lorenzi, R.3
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2
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49449097533
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Death receptor 5 mediated-apoptosis contributes to cholestatic liver disease
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The death signal mediated by TRAIL receptor 2/DR5 is a regulator of cholestatic liver injury and a likely therapeutic target
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Takeda K, Kojima Y, Ikejima K, et al. Death receptor 5 mediated-apoptosis contributes to cholestatic liver disease. Proc Natl Acad Sci U S A 2008; 105:10895-10900. The death signal mediated by TRAIL receptor 2/DR5 is a regulator of cholestatic liver injury and a likely therapeutic target.
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(2008)
Proc Natl Acad Sci U S A
, vol.105
, pp. 10895-10900
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Takeda, K.1
Kojima, Y.2
Ikejima, K.3
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3
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42249091320
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TRAIL mediates liver injury by the innate immune system in the bile duct-ligated mouse
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DOI 10.1002/hep.22136
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Kahraman A, Barreyro FJ, Bronk SF, et al. TRAIL mediates liver injury by the innate immune system in the bile duct-ligated mouse. Hepatology 2008; 47:1317-1330. (Pubitemid 351547918)
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(2008)
Hepatology
, vol.47
, Issue.4
, pp. 1317-1330
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Kahraman, A.1
Barreyro, F.J.2
Bronk, S.F.3
Werneburg, N.W.4
Mott, J.L.5
Akazawa, Y.6
Masuoka, H.C.7
Howe, C.L.8
Gores, G.J.9
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4
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49149090637
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Hepatocyte-specific ablation of Foxa2 alters bile acid homeostasis and results in endoplasmic reticulum stress
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Bochkis IM, Rubins NE, White P, et al. Hepatocyte-specific ablation of Foxa2 alters bile acid homeostasis and results in endoplasmic reticulum stress. Nat Med 2008; 14:828-836.
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(2008)
Nat Med
, vol.14
, pp. 828-836
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Bochkis, I.M.1
Rubins, N.E.2
White, P.3
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5
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38549109650
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Mechanisms of cholestasis
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vii. A very useful and timely review on biliary transporters and clinical disease
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Zollner G, Trauner M. Mechanisms of cholestasis. Clin Liver Dis 2008; 12:1-26; vii. A very useful and timely review on biliary transporters and clinical disease.
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(2008)
Clin Liver Dis
, vol.12
, pp. 1-26
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Zollner, G.1
Trauner, M.2
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6
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41549123313
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Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families
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A unique cohort of patients with BSEP deficiency described in both molecular and clinical detail. Important observation about the risk of hepatobiliary malignancy in these patients that appears to relate to the severity of the mutation
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Strautnieks SS, Byrne JA, Pawlikowska L, et al. Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families. Gastroenterology 2008; 134:1203-1214. A unique cohort of patients with BSEP deficiency described in both molecular and clinical detail. Important observation about the risk of hepatobiliary malignancy in these patients that appears to relate to the severity of the mutation.
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(2008)
Gastroenterology
, vol.134
, pp. 1203-1214
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Strautnieks, S.S.1
Byrne, J.A.2
Pawlikowska, L.3
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7
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46049094386
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ABCB4 Heterozygous Gene Mutations Associated with Fibrosing Cholestatic Liver Disease in Adults
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DOI 10.1053/j.gastro.2008.03.044, PII S0016508508005386
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Ziol M, Barbu V, Rosmorduc O, et al. ABCB4 heterozygous gene mutations associated with fibrosing cholestatic liver disease in adults. Gastroenterology 2008; 135:131-141. A valuable description of a molecular cause for hitherto cryptogenic cholestatic liver disease, reminding clinicians that there remains room for significant improvement in describing the cause of liver diseases. (Pubitemid 351899020)
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(2008)
Gastroenterology
, vol.135
, Issue.1
, pp. 131-141
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Ziol, M.1
Barbu, V.2
Rosmorduc, O.3
Frassati-Biaggi, A.4
Barget, N.5
Hermelin, B.6
Scheffer, G.L.7
Bennouna, S.8
Trinchet, J.9
Beaugrand, M.10
Ganne-Carrie, N.11
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8
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54449098345
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A mutation in the canalicular phospholipid transporter gene, ABCB4, is associated with cholestasis, ductopenia, and cirrhosis in adults
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Gotthardt D, Runz H, Keitel V, et al. A mutation in the canalicular phospholipid transporter gene, ABCB4, is associated with cholestasis, ductopenia, and cirrhosis in adults. Hepatology 2008; 48:1157-1166.
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(2008)
Hepatology
, vol.48
, pp. 1157-1166
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Gotthardt, D.1
Runz, H.2
Keitel, V.3
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9
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58949098338
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Comment on biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis
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Walker LJ, Newton J, Jones DE, Bassendine MF. Comment on biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatology 2008; 49:337-338.
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(2008)
Hepatology
, vol.49
, pp. 337-338
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Walker, L.J.1
Newton, J.2
Jones, D.E.3
Bassendine, M.F.4
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10
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38849104576
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Combination of ursodeoxycholic acid and glucocorticoids upregulates the AE2 alternate promoter in human liver cells
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Combination therapy for PBC with UDCA and steroids has been suggested. This study elegantly identifies molecular mechanisms that could contribute biologically to such a pharmacological approach, and identifies a biliary transporter as relevant therefore to both disease etiopathogenesis and treatment
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Arenas F, Hervias I, Uriz M, et al. Combination of ursodeoxycholic acid and glucocorticoids upregulates the AE2 alternate promoter in human liver cells. J Clin Invest 2008; 118:695-709. Combination therapy for PBC with UDCA and steroids has been suggested. This study elegantly identifies molecular mechanisms that could contribute biologically to such a pharmacological approach, and identifies a biliary transporter as relevant therefore to both disease etiopathogenesis and treatment.
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(2008)
J Clin Invest
, vol.118
, pp. 695-709
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Arenas, F.1
Hervias, I.2
Uriz, M.3
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11
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43049094474
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Ae2a,b-deficient mice develop antimitochondrial antibodies and other features resembling primary biliary cirrhosis
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Until recently, there were few animal models of PBC. This knockout mouse (AE2) adds to the growing list of murine genetic changes that lead to autoimmune cholangitis
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Salas JT, Banales JM, Sarvide S, et al. Ae2a,b-deficient mice develop antimitochondrial antibodies and other features resembling primary biliary cirrhosis. Gastroenterology 2008; 134:1482-1493. Until recently, there were few animal models of PBC. This knockout mouse (AE2) adds to the growing list of murine genetic changes that lead to autoimmune cholangitis.
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(2008)
Gastroenterology
, vol.134
, pp. 1482-1493
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Salas, J.T.1
Banales, J.M.2
Sarvide, S.3
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12
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34248669615
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Bile ductules and stromal cells express hedgehog ligands and/or hedgehog target genes in primary biliary cirrhosis
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DOI 10.1002/hep.21660
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Jung Y, McCall SJ, Li YX, Diehl AM. Bile ductules and stromal cells express hedgehog ligands and/or hedgehog target genes in primary biliary cirrhosis. Hepatology 2007; 45:1091-1096. (Pubitemid 46775770)
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(2007)
Hepatology
, vol.45
, Issue.5
, pp. 1091-1096
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Jung, Y.1
McCall, S.J.2
Li, Y.-X.3
Diehl, A.M.4
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13
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50249151525
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The hedgehog pathway regulates remodelling responses to biliary obstruction in rats
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Omenetti A, Popov Y, Jung Y, et al. The hedgehog pathway regulates remodelling responses to biliary obstruction in rats. Gut 2008; 57:1275-1282.
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(2008)
Gut
, vol.57
, pp. 1275-1282
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Omenetti, A.1
Popov, Y.2
Jung, Y.3
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14
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55849135600
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Hedgehog signaling regulates epithelial-mesenchymal transition during biliary fibrosis in rodents and humans
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This group has championed the role of the Hh signaling pathway in biliary fibrosis. This study now links the pathway to EMT in biliary fibrosis, another pathological mechanism proposed to be relevant to cholestatic liver disease
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Omenetti A, Porrello A, Jung Y, et al. Hedgehog signaling regulates epithelial-mesenchymal transition during biliary fibrosis in rodents and humans. J Clin Invest 2008; 118:3331-3342. This group has championed the role of the Hh signaling pathway in biliary fibrosis. This study now links the pathway to EMT in biliary fibrosis, another pathological mechanism proposed to be relevant to cholestatic liver disease.
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(2008)
J Clin Invest
, vol.118
, pp. 3331-3342
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Omenetti, A.1
Porrello, A.2
Jung, Y.3
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15
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34247334927
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Biliary epithelial-mesenchymal transition in posttransplantation recurrence of primary biliary cirrhosis
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DOI 10.1002/hep.21624
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Robertson H, Kirby JA, Yip WW, et al. Biliary epithelial-mesenchymal transition in posttransplantation recurrence of primary biliary cirrhosis. Hepatology 2007; 45:977-981. (Pubitemid 46631566)
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(2007)
Hepatology
, vol.45
, Issue.4
, pp. 977-981
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Robertson, H.1
Kirby, J.A.2
Yip, W.W.3
Jones, D.E.J.4
Burt, A.D.5
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16
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38549143883
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Epithelial-mesenchymal transition contributes to portal tract fibrogenesis during human chronic liver disease
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DOI 10.1038/labinvest.3700704, PII 3700704
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Rygiel KA, Robertson H, Marshall HL, et al. Epithelial-mesenchymal transition contributes to portal tract fibrogenesis during human chronic liver disease. Lab Invest 2008; 88:112-123. (Pubitemid 351161272)
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(2008)
Laboratory Investigation
, vol.88
, Issue.2
, pp. 112-123
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Rygiel, K.A.1
Robertson, H.2
Marshall, H.L.3
Pekalski, M.4
Zhao, L.5
Booth, T.A.6
Jones, D.E.J.7
Burt, A.D.8
Kirby, J.A.9
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17
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48549103201
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Inhibition of integrin alphavbeta6 on cholangiocytes blocks transforming growth factor-beta activation and retards biliary fibrosis progression
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A nice study demonstrating that inhibition of integrin alphavbeta6 may be of value to those with progressive biliary diseases
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Patsenker E, Popov Y, Stickel F, et al. Inhibition of integrin alphavbeta6 on cholangiocytes blocks transforming growth factor-beta activation and retards biliary fibrosis progression. Gastroenterology 2008; 135:660-670. A nice study demonstrating that inhibition of integrin alphavbeta6 may be of value to those with progressive biliary diseases.
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(2008)
Gastroenterology
, vol.135
, pp. 660-670
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Patsenker, E.1
Popov, Y.2
Stickel, F.3
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18
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39549107358
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Natural killer T cells exacerbate liver injury in a transforming growth factor beta receptor II dominant-negative mouse model of primary biliary cirrhosis
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Chuang YH, Lian ZX, Yang GX, et al. Natural killer T cells exacerbate liver injury in a transforming growth factor beta receptor II dominant-negative mouse model of primary biliary cirrhosis. Hepatology 2008; 47:571-580.
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(2008)
Hepatology
, vol.47
, pp. 571-580
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Chuang, Y.H.1
Lian, Z.X.2
Yang, G.X.3
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19
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49649091998
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Loss of tolerance in C57BL/6 mice to the autoantigen E2 subunit of pyruvate dehydrogenase by a xenobiotic with ensuing biliary ductular disease
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Demonstration that loss of tolerance immunologically to pyruvate dehydrogenase can occur with a xenobiotic and lead to a murine autoimmune cholangitis
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Wakabayashi K, Lian ZX, Leung PS, et al. Loss of tolerance in C57BL/6 mice to the autoantigen E2 subunit of pyruvate dehydrogenase by a xenobiotic with ensuing biliary ductular disease. Hepatology 2008; 48:531-540. Demonstration that loss of tolerance immunologically to pyruvate dehydrogenase can occur with a xenobiotic and lead to a murine autoimmune cholangitis.
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(2008)
Hepatology
, vol.48
, pp. 531-540
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Wakabayashi, K.1
Lian, Z.X.2
Leung, P.S.3
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20
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46249110385
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Adoptive transfer of CD8(+) T cells from transforming growth factor beta receptor type II (dominant negative form) induces autoimmune cholangitis in mice
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Yang GX, Lian ZX, Chuang YH, et al. Adoptive transfer of CD8(+) T cells from transforming growth factor beta receptor type II (dominant negative form) induces autoimmune cholangitis in mice. Hepatology 2008; 47:1974-1982.
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(2008)
Hepatology
, vol.47
, pp. 1974-1982
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Yang, G.X.1
Lian, Z.X.2
Chuang, Y.H.3
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21
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39549119353
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Interacting alleles of the coinhibitory immunoreceptor genes cytotoxic T-lymphocyte antigen 4 and programmed cell-death 1 influence risk and features of primary biliary cirrhosis
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Juran BD, Atkinson EJ, Schlicht EM, et al. Interacting alleles of the coinhibitory immunoreceptor genes cytotoxic T-lymphocyte antigen 4 and programmed cell-death 1 influence risk and features of primary biliary cirrhosis. Hepatology 2008; 47:563-570.
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(2008)
Hepatology
, vol.47
, pp. 563-570
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Juran, B.D.1
Atkinson, E.J.2
Schlicht, E.M.3
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22
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53049084465
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Primary biliary cirrhosis is associated with a genetic variant in the 3′ flanking region of the CTLA4 gene
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Demonstration that PBC has a genetic association with the immunoregulatory gene CTLA-4
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Juran BD, Atkinson EJ, Schlicht EM, et al. Primary biliary cirrhosis is associated with a genetic variant in the 3′ flanking region of the CTLA4 gene. Gastroenterology 2008; 135:1200-1206. Demonstration that PBC has a genetic association with the immunoregulatory gene CTLA-4.
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(2008)
Gastroenterology
, vol.135
, pp. 1200-1206
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Juran, B.D.1
Atkinson, E.J.2
Schlicht, E.M.3
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23
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58149376547
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Human leukocyte antigen polymorphisms in Italian primary biliary cirrhosis: A multicenter study of 664 patients and 1992 healthy controls
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A large study reinforcing the association between PBC and the human leukocyte antigen system. This paper additionally serves as a reminder of the appropriate study size needed to address genetic causes of multifactorial diseases, which for relatively rare diseases such as those seen in cholestatic liver disease, requires collaboration
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Invernizzi P, Selmi C, Poli F, et al. Human leukocyte antigen polymorphisms in Italian primary biliary cirrhosis: a multicenter study of 664 patients and 1992 healthy controls. Hepatology 2008; 48:1906-1912. A large study reinforcing the association between PBC and the human leukocyte antigen system. This paper additionally serves as a reminder of the appropriate study size needed to address genetic causes of multifactorial diseases, which for relatively rare diseases such as those seen in cholestatic liver disease, requires collaboration.
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(2008)
Hepatology
, vol.48
, pp. 1906-1912
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Invernizzi, P.1
Selmi, C.2
Poli, F.3
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24
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51349157245
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Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis
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A useful study for clinicians and patients identifying biochemical predictors of long-term outcome for patients with PBC. Normal liver biochemistry after 1 year of treatment with UDCA is associated with an excellent outcome for individuals.
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Corpechot C, Abenavoli L, Rabahi N, et al. Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatology 2008; 48:871-877. A useful study for clinicians and patients identifying biochemical predictors of long-term outcome for patients with PBC. Normal liver biochemistry after 1 year of treatment with UDCA is associated with an excellent outcome for individuals.
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(2008)
Hepatology
, vol.48
, pp. 871-877
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Corpechot, C.1
Abenavoli, L.2
Rabahi, N.3
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25
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33644854254
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Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid
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Pares A, Caballeria L, Rodes J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid. Gastroenterology 2006; 130:715-720.
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(2006)
Gastroenterology
, vol.130
, pp. 715-720
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Pares, A.1
Caballeria, L.2
Rodes, J.3
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26
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55249105177
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Portal hypertension and primary biliary cirrhosis: Effect of long-term ursodeoxycholic acid treatment
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A unique long-term study published that looked at portal hypertension serially in patients with PBC treated with UDCA. 'Responders' (stable or improved PHG and normalized AST level at 2 years) had a 15-year survival similar to healthy controls
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Huet PM, Vincent C, Deslaurier J, et al. Portal hypertension and primary biliary cirrhosis: effect of long-term ursodeoxycholic acid treatment. Gastroenterology 2008; 135:1552-1560. A unique long-term study published that looked at portal hypertension serially in patients with PBC treated with UDCA. 'Responders' (stable or improved PHG and normalized AST level at 2 years) had a 15-year survival similar to healthy controls.
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(2008)
Gastroenterology
, vol.135
, pp. 1552-1560
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Huet, P.M.1
Vincent, C.2
Deslaurier, J.3
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27
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56149116412
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Prediction of clinical outcomes in primary biliary cirrhosis by serum enhanced liver fibrosis assay
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Mayo MJ, Parkes J, Adams-Huet B, et al. Prediction of clinical outcomes in primary biliary cirrhosis by serum enhanced liver fibrosis assay. Hepatology 2008; 48:1549-1557.
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(2008)
Hepatology
, vol.48
, pp. 1549-1557
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Mayo, M.J.1
Parkes, J.2
Adams-Huet, B.3
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28
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42249106196
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Mortality attributable to cholestatic liver disease in the United States
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A reminder, if needed, that the greatest challenge in cholestatic liver disease is to change the natural history of sclerosing cholangitis. As compared with PBC in which mortality is falling, mortality for PSC remains disturbingly untouched
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Mendes FD, Kim WR, Pedersen R, et al. Mortality attributable to cholestatic liver disease in the United States. Hepatology 2008; 47:1241-1247. A reminder, if needed, that the greatest challenge in cholestatic liver disease is to change the natural history of sclerosing cholangitis. As compared with PBC in which mortality is falling, mortality for PSC remains disturbingly untouched.
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(2008)
Hepatology
, vol.47
, pp. 1241-1247
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Mendes, F.D.1
Kim, W.R.2
Pedersen, R.3
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29
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43149104736
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Incidence and mortality of primary sclerosing cholangitis in the UK: A population-based cohort study
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Card TR, Solaymani-Dodaran M, West J. Incidence and mortality of primary sclerosing cholangitis in the UK: a population-based cohort study. J Hepatol 2008; 48:939-944.
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(2008)
J Hepatol
, vol.48
, pp. 939-944
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Card, T.R.1
Solaymani-Dodaran, M.2
West, J.3
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30
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41349103851
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The natural history of small-duct primary sclerosing cholangitis
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A unique collection of patients from across the world with small-duct PSC demonstrating how about one in five progresses to large-duct disease over about 7 years. Cholangiocarcinoma was only seen in an individual who progressed
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Bjornsson E, Olsson R, Bergquist A, et al. The natural history of small-duct primary sclerosing cholangitis. Gastroenterology 2008; 134:975-980. A unique collection of patients from across the world with small-duct PSC demonstrating how about one in five progresses to large-duct disease over about 7 years. Cholangiocarcinoma was only seen in an individual who progressed.
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(2008)
Gastroenterology
, vol.134
, pp. 975-980
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Bjornsson, E.1
Olsson, R.2
Bergquist, A.3
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31
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42449114097
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A double-blind, placebo-controlled, randomized study of infliximab in primary sclerosing cholangitis
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DOI 10.1097/MCG.0b013e3181662426
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Hommes DW, Erkelens W, Ponsioen C, et al. A double-blind, placebo-controlled, randomized study of infliximab in primary sclerosing cholangitis. J Clin Gastroenterol 2008; 42:522-526. (Pubitemid 351572276)
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(2008)
Journal of Clinical Gastroenterology
, vol.42
, Issue.5
, pp. 522-526
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Hommes, D.W.1
Erkelens, W.2
Ponsioen, C.3
Stokkers, P.4
Rauws, E.5
Van Der Spek, M.6
Kate, F.T.7
Van Deventer, S.J.8
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32
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38649091144
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Immunoglobulin G4-associated cholangitis: Clinical profile and response to therapy
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A useful clinic description of IgG4-associated autoimmune pancreatitis/sclerosing cholangitis, demonstrating therapeutic responses and clinical outcomes
-
Ghazale A, Chari ST, Zhang L, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 2008; 134:706-715. A useful clinic description of IgG4-associated autoimmune pancreatitis/sclerosing cholangitis, demonstrating therapeutic responses and clinical outcomes.
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(2008)
Gastroenterology
, vol.134
, pp. 706-715
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Ghazale, A.1
Chari, S.T.2
Zhang, L.3
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