biliary liver cirrhosis

Summary

Summary: FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cirrhosis involves the destruction of small intra-hepatic bile ducts and bile secretion. Secondary biliary cirrhosis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes.

Top Publications

  1. ncbi The causes of primary biliary cirrhosis: Convenient and inconvenient truths
    M Eric Gershwin
    Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, CA 95616, USA
    Hepatology 47:737-45. 2008
  2. ncbi Primary biliary cirrhosis
    Marshall M Kaplan
    Division of Gastroenterology, Tufts New England Medical Center, Boston, MA 02111, USA
    N Engl J Med 353:1261-73. 2005
  3. pmc Risk factors and comorbidities in primary biliary cirrhosis: a controlled interview-based study of 1032 patients
    M Eric Gershwin
    Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, CA 95616, USA
    Hepatology 42:1194-202. 2005
  4. pmc Combination of ursodeoxycholic acid and glucocorticoids upregulates the AE2 alternate promoter in human liver cells
    Fabián Arenas
    Division of Gene Therapy and Hepatology, Center for Applied Medical Research, Clinica Universitaria, University of Navarra School of Medicine, CIBERehd, Pamplona, Spain
    J Clin Invest 118:695-709. 2008
  5. pmc Prevalence and clinical significance of isotype specific antinuclear antibodies in primary biliary cirrhosis
    E I Rigopoulou
    Institute of Liver Studies, King s College Hospital, Denmark Hill, London SE5 9RS, UK
    Gut 54:528-32. 2005
  6. ncbi Anti-gp210 and anti-centromere antibodies are different risk factors for the progression of primary biliary cirrhosis
    Minoru Nakamura
    Clinical Research Center, National Hospital Organization NHO Nagasaki Medical Center and Department of Hepatology, Nagasaki University Graduate School of Biomedical Sciences, Omura, Nagasaki, Japan
    Hepatology 45:118-27. 2007
  7. ncbi Primary biliary cirrhosis once rare, now common in the United Kingdom?
    O F James
    Centre for Liver Research, University of Newcastle, The Medical School, Framlington Place, Newcastle upon Tyne, UK
    Hepatology 30:390-4. 1999
  8. pmc Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants
    Gideon M Hirschfield
    University of Toronto and Liver Center, Toronto Western Hospital, Toronto, ON, Canada
    N Engl J Med 360:2544-55. 2009
  9. ncbi Primary biliary cirrhosis following Lactobacillus vaccination for recurrent vaginitis
    Dimitrios Bogdanos
    Institute of Liver Studies, King s College, London School of Medicine at King s College Hospital, London, United Kingdom
    J Hepatol 49:466-73. 2008
  10. ncbi Primary biliary cirrhosis specific antinuclear antibodies in patients from Spain
    Dimitrios Petrou Bogdanos
    Am J Gastroenterol 99:763-4; author reply 765. 2004

Research Grants

Detail Information

Publications246 found, 100 shown here

  1. ncbi The causes of primary biliary cirrhosis: Convenient and inconvenient truths
    M Eric Gershwin
    Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, CA 95616, USA
    Hepatology 47:737-45. 2008
    ..We present these data under the umbrella of convenient truths that support this thesis as well as some inconvenient truths that are not readily accommodated by current theory...
  2. ncbi Primary biliary cirrhosis
    Marshall M Kaplan
    Division of Gastroenterology, Tufts New England Medical Center, Boston, MA 02111, USA
    N Engl J Med 353:1261-73. 2005
  3. pmc Risk factors and comorbidities in primary biliary cirrhosis: a controlled interview-based study of 1032 patients
    M Eric Gershwin
    Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, CA 95616, USA
    Hepatology 42:1194-202. 2005
    ..Exogenous estrogens may also contribute to explain the female predominance of the disease...
  4. pmc Combination of ursodeoxycholic acid and glucocorticoids upregulates the AE2 alternate promoter in human liver cells
    Fabián Arenas
    Division of Gene Therapy and Hepatology, Center for Applied Medical Research, Clinica Universitaria, University of Navarra School of Medicine, CIBERehd, Pamplona, Spain
    J Clin Invest 118:695-709. 2008
    ..Our data provide a potential molecular explanation for the beneficial effects of the combination of UDCA and glucocorticoids in PBC patients with inadequate response to UDCA monotherapy...
  5. pmc Prevalence and clinical significance of isotype specific antinuclear antibodies in primary biliary cirrhosis
    E I Rigopoulou
    Institute of Liver Studies, King s College Hospital, Denmark Hill, London SE5 9RS, UK
    Gut 54:528-32. 2005
    ..Antinuclear antibodies (ANA) giving a rim-like/membranous (RL/M) or a multiple nuclear dot (MND) pattern are highly specific for primary biliary cirrhosis (PBC). Aim and..
  6. ncbi Anti-gp210 and anti-centromere antibodies are different risk factors for the progression of primary biliary cirrhosis
    Minoru Nakamura
    Clinical Research Center, National Hospital Organization NHO Nagasaki Medical Center and Department of Hepatology, Nagasaki University Graduate School of Biomedical Sciences, Omura, Nagasaki, Japan
    Hepatology 45:118-27. 2007
    ....
  7. ncbi Primary biliary cirrhosis once rare, now common in the United Kingdom?
    O F James
    Centre for Liver Research, University of Newcastle, The Medical School, Framlington Place, Newcastle upon Tyne, UK
    Hepatology 30:390-4. 1999
    ..The study draws attention to (1) high mortality from liver disease and non-liver-related causes even in patients initially with no liver symptoms and (2) apparently poor diagnostic awareness of the disease...
  8. pmc Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants
    Gideon M Hirschfield
    University of Toronto and Liver Center, Toronto Western Hospital, Toronto, ON, Canada
    N Engl J Med 360:2544-55. 2009
    ..Twin and family aggregation data suggest that there is a significant genetic predisposition to primary biliary cirrhosis, but the susceptibility loci are unknown...
  9. ncbi Primary biliary cirrhosis following Lactobacillus vaccination for recurrent vaginitis
    Dimitrios Bogdanos
    Institute of Liver Studies, King s College, London School of Medicine at King s College Hospital, London, United Kingdom
    J Hepatol 49:466-73. 2008
    ..We report a case of symptomatic PBC diagnosed after sequential immunization with a lactobacillus vaccine for recurrent vaginitis over years...
  10. ncbi Primary biliary cirrhosis specific antinuclear antibodies in patients from Spain
    Dimitrios Petrou Bogdanos
    Am J Gastroenterol 99:763-4; author reply 765. 2004
  11. ncbi Autoantibodies against nuclear pore complexes are associated with more active and severe liver disease in primary biliary cirrhosis
    P Invernizzi
    Department of Medicine, Surgery and Dentistry, San Paolo School of Medicine, University of Milan, Italy
    J Hepatol 34:366-72. 2001
    ..Antibodies against nuclear pore complexes (NPCs) have been detected in primary biliary cirrhosis (PBC), but their clinical relevance is still unsettled...
  12. pmc Human leukocyte antigen polymorphisms in Italian primary biliary cirrhosis: a multicenter study of 664 patients and 1992 healthy controls
    Pietro Invernizzi
    Department of Internal Medicine, Istituto Clinico Humanitas IRCCS, University of Milan, Milan, Italy
    Hepatology 48:1906-12. 2008
    ..We suggest that a large whole-genome approach is required to identify further genetic elements contributing to the loss of tolerance in this disease...
  13. ncbi Profile and clinical significance of anti-nuclear envelope antibodies found in patients with primary biliary cirrhosis: a multicenter study
    Kiyomitsu Miyachi
    First Diagnostic Division, Health Sciences Research Institute Inc, 106 Godo cho Hodogaya ku, Yokohama shi, Kanagawa 240 0005, Japan
    J Autoimmun 20:247-54. 2003
    ..This difference was found to be statistically significant. The presence of anti-p62 complex antibody may be related with the progressive or advanced state of PBC...
  14. ncbi Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis
    Christophe Corpechot
    Hepatology Department, Reference Center for Inflammatory Biliary Diseases, Saint Antoine Hospital, National Institute of Health and Medical Research INSERM, Pierre et Marie Curie University Paris 6, France
    Hepatology 48:871-7. 2008
    ..3). Antinuclear antibodies against gp210 or Sp100 proteins were associated with death or LT in univariate but not in multivariate analysis...
  15. pmc Identification of novel molecules and pathogenic pathways in primary biliary cirrhosis: cDNA array analysis of intrahepatic differential gene expression
    N A Shackel
    AW Morrow Gastroenterology and Liver Centre, Centenary Institute of Cancer Medicine and Cell Biology, Royal Prince Alfred Hospital and the University of Sydney, Sydney, Australia
    Gut 49:565-76. 2001
    ..CONCLUSION: Many genes implicated in intrahepatic inflammation, fibrosis, and regeneration were upregulated in PBC cirrhosis. In particular, increased expression of a number of Drosophila homologues was seen in PBC...
  16. ncbi Budesonide combined with UDCA to improve liver histology in primary biliary cirrhosis: a three-year randomized trial
    Henna Rautiainen
    Department of Medicine, Division of Gastroenterology, Helsinki University Central Hospital, 00029 HYKS, Finland
    Hepatology 41:747-52. 2005
    ..Studies with longer follow-up using a combination of budesonide and UDCA are warranted to confirm safety and effects...
  17. ncbi IL-2 receptor alpha(-/-) mice and the development of primary biliary cirrhosis
    Kanji Wakabayashi
    Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis, Davis, CA, USA
    Hepatology 44:1240-9. 2006
    ....
  18. ncbi Autoreactive T-cell responses in primary biliary cirrhosis are proinflammatory whereas those of controls are regulatory
    Shinji Shimoda
    Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan
    Gastroenterology 131:606-18. 2006
    ..Understanding the mechanism for activation of autoreactive T cells will be critical in the treatment of autoimmune diseases...
  19. ncbi B cells suppress the inflammatory response in a mouse model of primary biliary cirrhosis
    Yuki Moritoki
    Division of Rheumatology, Allergy and Clinical Immunology, University of California, Davis, California, USA
    Gastroenterology 136:1037-47. 2009
    ..Mice that express a dominant-negative form of transforming growth factor-beta receptor restricted to T cells (dnTGF-betaRII) develop antimitochondrial antibodies and liver inflammation similar to human primary biliary cirrhosis...
  20. ncbi Management of primary biliary cirrhosis. The American Association for the Study of Liver Diseases practice guidelines
    E J Heathcote
    Division of Gastroenterology, University of Toronto, The Toronto Hospital, Toronto, Ontario, Canada
    Hepatology 31:1005-13. 2000
    ..PBC is diagnosed with increasing frequency, but the agent(s) responsible for this slowly progressive destruction of the interlobular bile ducts remains elusive and hence a specific therapy remains unavailable...
  21. ncbi IL-2 receptor alpha deficiency and features of primary biliary cirrhosis
    Christopher A Aoki
    Division of Gastroenterology, University of California at Davis, 95616, USA
    J Autoimmun 27:50-3. 2006
    ..This case illustrates the role of deficiency of CD4+CD25+ Treg cells in causing autoimmunity, and speaks to the potential use of allogenic stem cell transplantation for immunoreconstitution in adult PBC...
  22. ncbi Survival and symptom progression in a geographically based cohort of patients with primary biliary cirrhosis: follow-up for up to 28 years
    Martin Prince
    Centre for Liver Research, The Medical School, Framlington Place, Newcastle upon Tyne, United Kingdom
    Gastroenterology 123:1044-51. 2002
    ..We examined the prognosis of primary biliary cirrhosis in a comprehensive geographically defined cohort...
  23. ncbi Primary biliary cirrhosis: an infectious disease caused by Chlamydia pneumoniae?
    Ahmad S Abdulkarim
    Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    J Hepatol 40:380-4. 2004
    ..Both an infectious etiology and molecular mimicry have been implicated. The aim is to study the prevalence of Chlamydial antigens and RNA in the liver tissue of patients with PBC...
  24. ncbi Chemical xenobiotics and mitochondrial autoantigens in primary biliary cirrhosis: identification of antibodies against a common environmental, cosmetic, and food additive, 2-octynoic acid
    Katsushi Amano
    Division of Rheumatology, Allergy and Clinical Immunology, Genomic and Biomedical Sciences Facility, University of California, Davis, CA 95616, USA
    J Immunol 174:5874-83. 2005
    ..Not only does 2-octynoic acid have the potential to modify PDC-E2 in vivo but importantly it was/is widely used in the environment including perfumes, lipstick, and many common food flavorings...
  25. pmc Is there a relation between Chlamydia infection and primary biliary cirrhosis?
    Patrick S C Leung
    Division of Rheumatology, Allergy and Clinical Immunology, University of California, Davis, CA 95616, USA
    Clin Dev Immunol 10:227-33. 2003
    ..The detection of Chlamydia specific antibodies but not Chlamydia rRNA gene and Chlamydia antigens in PBC suggests that Chlamydia infection is not involved in PBC...
  26. ncbi Ae2a,b-deficient mice develop antimitochondrial antibodies and other features resembling primary biliary cirrhosis
    January T Salas
    Division of Gene Therapy and Hepatology, CIMA, Clinica Universitaria and School of Medicine, University of Navarra, and CIBEREHD, Pamplona, Spain
    Gastroenterology 134:1482-93. 2008
    ..In mice with widespread Ae2 gene disruption, we previously reported altered spermiogenesis and reduced gastric acid secretion. We now describe the hepatobiliary and immunologic changes observed in these Ae2(a.b)-deficient mice...
  27. ncbi Primary biliary cirrhosis in monozygotic and dizygotic twins: genetics, epigenetics, and environment
    Carlo Selmi
    Division of Rheumatology, Allergy, and Clinical Immunology, University of California at Davis, 95616, USA
    Gastroenterology 127:485-92. 2004
    ..Accordingly, we evaluated the concordance of PBC in a genetically defined population of twin sets and evaluated the clinical characteristics between concordant subjects...
  28. ncbi Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic Acid
    Albert Pares
    Liver Unit, Digestive Diseases Institute, Hospital Clinic, IDIBAPS, Barcelona, Spain
    Gastroenterology 130:715-20. 2006
    ....
  29. ncbi Cloning the human betaretrovirus proviral genome from patients with primary biliary cirrhosis
    Lizhe Xu
    Section of Gastroenterology and Hepatology, Ochsner Clinic Foundation, New Orleans, LA, USA
    Hepatology 39:151-6. 2004
    ..In conclusion, human betaretrovirus nucleic acid sequences have been cloned from patients with PBC. They share marked homology with MMTV and human breast cancer-derived retrovirus sequences...
  30. ncbi The epidemiology of primary biliary cirrhosis
    Martin I Prince
    Centre for Liver Research, William Leech Building, Newcastle Medical School, Framlington Place, Newcastle NE2 4HH, UK
    Clin Liver Dis 7:795-819. 2003
    ..These need repeating in new settings. We suggest that, as with other diseases, modern epidemiological instruments used in well-designed studies may provide important clues to the cause or causes of this disease...
  31. ncbi Improved prognosis of patients with primary biliary cirrhosis that have a biochemical response to ursodeoxycholic acid
    Edith M M Kuiper
    Department of Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, The Netherlands
    Gastroenterology 136:1281-7. 2009
    ..We performed a prospective multicenter study of patients with PBC treated with UDCA to compare prognosis with biochemical response...
  32. ncbi Patterns of autoimmunity in primary biliary cirrhosis patients and their families: a population-based cohort study
    F E Watt
    Centre for Liver Research, University of Newcastle, Newcastle upon Tyne, UK
    QJM 97:397-406. 2004
    ..Aim: To examine the prevalence and association patterns of autoimmune disease in a representative group of PBC patients...
  33. ncbi Hepatocellular carcinoma in primary biliary cirrhosis: similar incidence to that in hepatitis C virus-related cirrhosis
    L Caballeria
    Digestive Diseases Institute, Hospital Clinic, IDIBAPS, University of Barcelona, Spain
    Am J Gastroenterol 96:1160-3. 2001
    ..The prevalence of hepatocellular carcinoma (HCC) in primary biliary cirrhosis (PBC) is not well established, as some reports suggest a low risk, whereas others indicate that HCC may be no less frequent than in other types of cirrhosis...
  34. ncbi Identification and specificity of a cDNA encoding the 70 kd mitochondrial antigen recognized in primary biliary cirrhosis
    M E Gershwin
    J Immunol 138:3525-31. 1987
    ..The availability of a recombinant form of the 70 kd mitochondrial autoantigen will allow several definitive questions to be addressed in PBC, including identification of B cell epitopes, T cell recognition, and a model of PBC in mice...
  35. ncbi Combined analysis of the effect of treatment with ursodeoxycholic acid on histologic progression in primary biliary cirrhosis
    Renee Eugenie Poupon
    Inserm Unit 370, Faculte de Medecine Necker, 156 rue de Vaugirard, 75730 Paris Cedex 15, France
    J Hepatol 39:12-6. 2003
    ..This study aimed at evaluating the effect of ursodeoxycholic acid (UDCA) treatment on histologic progression in primary biliary cirrhosis (PBC)...
  36. ncbi The geographical distribution of primary biliary cirrhosis in a well-defined cohort
    M I Prince
    Centre for Liver Research, Newcastle Medical School, Framlington Place, Newcastle, England, UK
    Hepatology 34:1083-8. 2001
    ..PBC occurred to a density of 10.7 cases/km(2) in the highest risk areas. In conclusion, PBC is unevenly distributed in Northeast England. This may reflect one or more environmental risk factors in its etiology...
  37. ncbi The changing clinical presentation of recurrent primary biliary cirrhosis after liver transplantation
    Edmund Q Sanchez
    Baylor University Medical Center, Transplantation Services, Dallas, TX 75246, USA
    Transplantation 76:1583-8. 2003
    ..Recurrent primary biliary cirrhosis (RPBC) is a histologic diagnosis. Clinical data is unreliable in predicting or diagnosing recurrence. RPBC appears to have a changing clinical presentation in recent years...
  38. ncbi Primary biliary cirrhosis with additional features of autoimmune hepatitis: response to therapy with ursodeoxycholic acid
    Supriya Joshi
    Department of Medicine, The University Health Network, University of Toronto, Toronto, Ontario, Canada
    Hepatology 35:409-13. 2002
    ..Survival was similar for patients with PBC with and without ANA. In conclusion, features of AIH in PBC may be transient and response to UDCA therapy similar to patients with PBC without features of AIH...
  39. ncbi Novosphingobium aromaticivorans: a potential initiator of primary biliary cirrhosis
    Marshall M Kaplan
    Am J Gastroenterol 99:2147-9. 2004
    ..These observations provide additional evidence that exposure to N. aromaticivorans may trigger the development of PBC...
  40. pmc Primary structure of the human M2 mitochondrial autoantigen of primary biliary cirrhosis: dihydrolipoamide acetyltransferase
    R L Coppel
    Walter and Eliza Hall Institute of Medical Research, Parkville, Victoria, Australia
    Proc Natl Acad Sci U S A 85:7317-21. 1988
    ..Expressed fragments of the cDNA react strongly with sera from patients with primary biliary cirrhosis but not with sera from patients with autoimmune chronic active hepatitis or sera from healthy subjects...
  41. ncbi Primary biliary cirrhosis: what we know and what we want to know about human PBC and spontaneous PBC mouse models
    Yoshiyuki Ueno
    Division of Gastroenterology, Tohoku University Graduate School of Medicine, 1 1 Seiryo, Aoba, Sendai, Miyagi, 980 8574, Japan
    J Gastroenterol 42:189-95. 2007
    ..In this brief review, we discuss what we know and we do not know regarding the pathogenesis of PBC...
  42. ncbi Identification and characterization of a leukocyte-specific component of the nuclear body
    D B Bloch
    Department of Medicine, Harvard Medical School, Massachusetts General Hospital, Boston, Massachusetts 02129, USA
    J Biol Chem 271:29198-204. 1996
    ..The location of Sp140 in the NB, and expression of this gene in cells involved in host defense, suggest that Sp140 may be involved in the pathogenesis of acute promyelocytic leukemia and viral infection...
  43. ncbi The effect of immunosuppressive regimens on the recurrence of primary biliary cirrhosis after liver transplantation
    Josh Levitsky
    Center for Liver Diseases, The University of Chicago Hospitals, IL 60637, USA
    Liver Transpl 9:733-6. 2003
    ..In conclusion, recurrent PBC developed in a small number of patients 2 years or longer after OLT. In our population, there was no difference in recurrence rates between those administered cyclosporine or tacrolimus for immunosuppression...
  44. ncbi Biliary epithelial-mesenchymal transition in posttransplantation recurrence of primary biliary cirrhosis
    Helen Robertson
    Applied Immunobiology and Transplantation Research Group, Newcastle University, Newcastle upon Tyne, NE2 4HH, UK
    Hepatology 45:977-81. 2007
    ..S100A4 expression by BEC appears to occur before the development of any other features of recurrent PBC, suggesting that EMT may be an initiating event...
  45. ncbi Characterization and clinical impact of antinuclear antibodies in primary biliary cirrhosis
    Paolo Muratori
    Dipartimento di Medicina Interna, Cardioangiologia, Epatologia, Alma Mater Studiorum, Universita di Bologna, Policlinico S Orsola Malpighi, Italy
    Am J Gastroenterol 98:431-7. 2003
    ..We analyzed in detail the antinuclear antibodies reactivity of primary biliary cirrhosis patients and correlated the fine specificities observed with clinical, biochemical, and immunologic parameters...
  46. ncbi Myeloperoxidase-positive inflammatory cells participate in bile duct damage in primary biliary cirrhosis through nitric oxide-mediated reactions
    Chih Te Wu
    Division of Rheumatology, Allergy and Clinical Immunology, School of Medicine, University of California at Davis, TB 192, Davis, CA 95616, USA
    Hepatology 38:1018-25. 2003
    ..In conclusion, NO and reactive oxygen species, collectively determined as 3-nitrotyrosine, are associated with bile duct destruction in PBC and are particularly prevalent in early stage disease...
  47. pmc Fatigue and primary biliary cirrhosis: association of globus pallidus magnetisation transfer ratio measurements with fatigue severity and blood manganese levels
    D M Forton
    Liver Unit, Faculty of Medicine, St Mary s Hospital Campus, Imperial College London, London, UK
    Gut 53:587-92. 2004
    ..We examined the hypothesis that a CNS abnormality related to cholestasis, rather than cirrhosis per se, underlies this symptom...
  48. ncbi Altered monocyte responses to defined TLR ligands in patients with primary biliary cirrhosis
    Tin Ky Mao
    Division of Rheumatology, Allergy and Clinical Immunology, University of California, Davis, CA 95616, USA
    Hepatology 42:802-8. 2005
    ....
  49. pmc Primary biliary cirrhosis is associated with altered hepatic microRNA expression
    Kerstien A Padgett
    Department of Medical Microbiology and Immunology, University of California, Davis, CA 95616, USA
    J Autoimmun 32:246-53. 2009
    ..Our data are the first to demonstrate that PBC is characterized by altered expression of hepatic miRNA; however additional studies are required to demonstrate a causal link between those miRNA and the development of PBC...
  50. ncbi Lack of immunological or molecular evidence for a role of mouse mammary tumor retrovirus in primary biliary cirrhosis
    Carlo Selmi
    Division of Rheumatology, Allergy, and Clinical Immunology, University of California at Davis, 95616, USA
    Gastroenterology 127:493-501. 2004
    ..Recent observations, including a pilot clinical trial, have suggested that a human mouse mammary tumor virus (MMTV) causes primary biliary cirrhosis (PBC). We attempted to confirm such data...
  51. pmc Antimitochondrial antibodies in acute liver failure: implications for primary biliary cirrhosis
    Patrick S C Leung
    Division of Rheumatology Allergy, University of California, Davis Medical Center, Sacramento, CA, USA
    Hepatology 46:1436-42. 2007
    ..The rapid disappearance of AMAs in these patients provides further support for the contention that PBC pathogenesis requires additional factors, including genetic susceptibility...
  52. pmc NOD.c3c4 congenic mice develop autoimmune biliary disease that serologically and pathogenetically models human primary biliary cirrhosis
    Junichiro Irie
    Division of Rheumatology and Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261, USA
    J Exp Med 203:1209-19. 2006
    ..Using a congenic mapping approach, we define the first ABD (Abd) locus, Abd1. These results identify the NOD.c3c4 mouse as the first spontaneous mouse model of PBC...
  53. ncbi Antifibrotic effect of silymarin in rat secondary biliary fibrosis is mediated by downregulation of procollagen alpha1(I) and TIMP-1
    J D Jia
    Department of Gastroenterology and Hepatology, Friedrich-Alexander University, Erlangen-Nuernberg, Erlangen, Germany
    J Hepatol 35:392-8. 2001
    ..The serum procollagen type III propeptide level mirrors profibrogenic mRNA expression in the liver...
  54. ncbi Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis
    Jozefa Wesierska-Gadek
    First Department of Internal Medicine, Institute of Cancer Research, Vienna Medical University, Vienna, Austria
    Hepatology 43:1135-44. 2006
    ..5%, P = .019). Anti-NPC antibodies remained stable or slightly increased over the period of observation. In condusion, anti-NPC identifies patients likely to experience an unfavorable clinical course and more rapid disease progression...
  55. ncbi Bacterial CpG induces hyper-IgM production in CD27(+) memory B cells in primary biliary cirrhosis
    Kentaro Kikuchi
    Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis, Davis, CA 95616, USA
    Gastroenterology 128:304-12. 2005
    ..We hypothesized that the increase in serum IgM is the result of chronic B-cell activation induced via the Toll-like receptor (TLR) signaling pathway...
  56. pmc Apotopes and the biliary specificity of primary biliary cirrhosis
    Ana Lleo
    Division of Rheumatology, University of California at Davis, Davis, CA 95616, USA
    Hepatology 49:871-9. 2009
    ..The fact that AMA-containing sera reacted with PDC-E2 on apoptotic BECs without a requirement for permeabilization suggests that the autoantigen is accessible to the immune system during apoptosis...
  57. pmc Does a betaretrovirus infection trigger primary biliary cirrhosis?
    Lizhe Xu
    Section of Gastroenterology and Hepatology, Ochsner Clinic Foundation, New Orleans, LA 70121, USA
    Proc Natl Acad Sci U S A 100:8454-9. 2003
    ....
  58. ncbi Frequency of monosomy X in women with primary biliary cirrhosis
    Pietro Invernizzi
    Department of Internal Medicine University of Milan, Via di Rudini 8, 20142 Milan, Italy
    Lancet 363:533-5. 2004
    ..050 (95% CI 0.046-0.055) in women with PBC, 0.032 (0.028-0.036) in those with chronic hepatitis C, and 0.028 (0.025-0.032) in controls. We suggest that haploinsufficiency for specific X-linked genes leads to female susceptibility to PBC...
  59. pmc Bcl-2-dependent oxidation of pyruvate dehydrogenase-E2, a primary biliary cirrhosis autoantigen, during apoptosis
    J A Odin
    Department of Medicine, The Mount Sinai School of Medicine, New York, New York 10029, USA
    J Clin Invest 108:223-32. 2001
    ..We postulate that apoptotic cholangiocytes, unlike other apoptotic cell types, are a potential source of immunogenic PDC-E2 in patients with PBC...
  60. ncbi Biliary epithelial cells and primary biliary cirrhosis: the role of liver-infiltrating mononuclear cells
    Shinji Shimoda
    Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Hepatology 47:958-65. 2008
    ..Furthermore, the addition of autologous LMNCs to PBC BECs led to the production of higher levels of chemokines and enhanced the expression of CD40 and HLA-DRalpha...
  61. ncbi Long-term efficacy of sertraline as a treatment for cholestatic pruritus in patients with primary biliary cirrhosis
    Jeff Browning
    Division of Digestive and Liver Diseases, Department of Internal Medicine, The University of Texas Southwestern Medical Center at Dallas, 5323 Harry Hines Boulevard, Dallas, TX 75390 9151, USA
    Am J Gastroenterol 98:2736-41. 2003
    ....
  62. ncbi Increased prevalence of primary biliary cirrhosis near Superfund toxic waste sites
    Aftab Ala
    Mount Sinai School of Medicine, New York, NY 10029, USA
    Hepatology 43:525-31. 2006
    ..Significant clusters of both PBC-OLT and PBC-MSSM were identified surrounding SFS. In conclusion, toxin exposure may be a risk factor influencing the clustering of PBC cases...
  63. ncbi Patients with primary biliary cirrhosis react against a ubiquitous xenobiotic-metabolizing bacterium
    Carlo Selmi
    Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, CA 95616, USA
    Hepatology 38:1250-7. 2003
    ..aromaticivorans is a candidate for the induction of PBC...
  64. ncbi Recurrence of primary biliary cirrhosis after liver transplantation: Histologic estimate of incidence and natural history
    Pamela B Sylvestre
    Division of Anatomic Pathology, Mayo Clinic and Foundation, Rochester, MN, USA
    Liver Transpl 9:1086-93. 2003
    ..7 years of follow-up. When criteria for histologic recurrence are expanded to include moderate lymphocytic cholangitis with lymphoplasmacytic portal infiltrate, the recurrence rate of PBC is estimated as 26%...
  65. ncbi Mycophenolate mofetil for the treatment of primary biliary cirrhosis in patients with an incomplete response to ursodeoxycholic acid
    Jayant A Talwalkar
    Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    J Clin Gastroenterol 39:168-71. 2005
    ..Mycophenolate mofetil (MMF) is an immunosuppressive medication that inhibits T and B lymphocyte proliferation. The aim of this investigation was to determine the safety and estimated efficacy of MMF in patients with PBC...
  66. ncbi [Analysis of HLA alleles polymorphism in Chinese patients with primary biliary cirrhosis]
    Hai Ying Liu
    Department of Laboratory Diagnostics, Changzheng Hospital, Second Military Medical University, Shanghai 200003, China
    Zhonghua Gan Zang Bing Za Zhi 13:410-3. 2005
    ....
  67. ncbi Expression of adhesion molecules on mature cholangiocytes in canal of Hering and bile ductules in wedge biopsy samples of primary biliary cirrhosis
    Hiroaki Yokomori
    Kitasato Institute Medical Center Hospital, 121 1 Arai, Kitamotoshi, Saitama 364 8501, Japan
    World J Gastroenterol 11:4382-9. 2005
    ....
  68. ncbi Immunosuppression affects the rate of recurrent primary biliary cirrhosis after liver transplantation
    James Neuberger
    Liver Unit, Queen Elizabeth Hospital, Birmingham, UK
    Liver Transpl 10:488-91. 2004
    ..For those receiving cyclosporine, the median time to recurrence was 123 months and for those on tacrolimus 62 months (P <.001). Reasons for this difference between the 2 calcineurin inhibitors are not clear...
  69. ncbi No relevant effect of ursodeoxycholic acid on cytochrome P450 3A metabolism in primary biliary cirrhosis
    Karin Dilger
    Dr Falk Pharma GmbH, Freiburg, Germany
    Hepatology 41:595-602. 2005
    ..g., AUC(16alpha-hydroxyprednisolone)/AUC(budesonide) in patients: baseline, 8.6 +/- 3.9; UDCA, 10.7 +/- 7.1; rifampicin, 527.0 +/- 248.7). In conclusion, UDCA is not a relevant inducer of CYP3A enzymes in humans...
  70. ncbi Retrovirus infection as a trigger for primary biliary cirrhosis?
    Raoul Poupon
    Service d Hepatologie, Hopital Saint Antoine, Assistance Publique Hopitaux de Paris, 75571 cedex 12, Paris, France
    Lancet 363:260-1. 2004
  71. ncbi Epidemiology and pathogenesis of primary biliary cirrhosis
    Carlo Selmi
    Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis, School of Medicine, Davis, CA 95616, USA
    J Clin Gastroenterol 38:264-71. 2004
    ..In this review, the available data regarding the epidemiology and pathogenesis of primary biliary cirrhosis will be described and discussed...
  72. ncbi Expression of intercellular adhesion molecule-1 and lymphocyte function-associated antigen-1 protein and messenger RNA in primary biliary cirrhosis
    Hiroaki Yokomori
    Department of Internal Medicine, Kitasato Institute Medical Center Hospital, Kitamotoshi, Saitama
    Intern Med 42:947-54. 2003
    ..This study examined the role of intercellular adhesion molecule-1 (ICAM-1) and lymphocyte function-associated antigen-1 (LFA-1) in the autoimmune process of bile duct destruction in the early stages of primary biliary cirrhosis (PBC)...
  73. ncbi Liver transplantation for primary biliary cirrhosis
    Gerry C MacQuillan
    Liver Unit, Queen Elizabeth Hospital, Birmingham, B15 2TH England, UK
    Clin Liver Dis 7:941-56, ix. 2003
    ..Following transplantation, antimitochondrial antibodies persist and histological features of recurrent PBC may be seen in the allograft in up to 50% by 10 years; however, at least in the medium-term, this rarely causes clinical problems...
  74. ncbi Measurement of gp210 autoantibodies in sera of patients with primary biliary cirrhosis
    Alicja Bauer
    Department of Biochemistry and Molecular Biology, Medical Center of Postgraduate Education, Warsaw, Poland
    J Clin Lab Anal 21:227-31. 2007
    ..The results were negative for healthy blood donors and other controls. The specificity of the test was 99%, so the anti-gp-210 autoantibodies estimated on DRKASPPSGLWSPAY can be a reliable marker of PBC...
  75. pmc Which patients with primary biliary cirrhosis or primary sclerosing cholangitis should undergo endoscopic screening for oesophageal varices detection?
    B Bressler
    Toronto Western Hospital, University Health Network, 399 Bathurst Street, 6th Fl Fell Pavilion Rm 170, Toronto, Ontario M5T 2S8, Canada
    Gut 54:407-10. 2005
    ....
  76. ncbi Quantitation and phenotypic analysis of natural killer T cells in primary biliary cirrhosis using a human CD1d tetramer
    Hiroto Kita
    Division of Rheumatology, Allergy, and Clinical Immunology, University of California, Davis, CA 95616, USA
    Gastroenterology 123:1031-43. 2002
    ..However, there have been only limited studies attempting to quantitate the number of NKT cells in autoimmune disease, particularly because of difficulties associated with definition of this subpopulation...
  77. pmc The genetic basis of primary biliary cirrhosis: premises, not promises
    Pietro Invernizzi
    Gastroenterology 135:1044-7. 2008
  78. ncbi Identification of 2-nonynoic acid, a cosmetic component, as a potential trigger of primary biliary cirrhosis
    Roman Rieger
    Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis, GBSF 6510, 95616, USA
    J Autoimmun 27:7-16. 2006
    ..Our findings illustrate an unusual polyreactivity of anti-PDC-E2 and support the idea of epitope mimicry in the genesis of this autoantibody and perhaps of PBC itself...
  79. ncbi Ursodeoxycholic acid for patients with primary biliary cirrhosis: an updated systematic review and meta-analysis of randomized clinical trials using Bayesian approach as sensitivity analyses
    Yan Gong
    The Cochrane Hepato Biliary Group, Copenhagen Trial Unit, Center for Clinical Intervention Research, Department 7102, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark
    Am J Gastroenterol 102:1799-807. 2007
    ..Ursodeoxycholic acid (UDCA) is used for primary biliary cirrhosis (PBC), but the beneficial effects remain controversial...
  80. pmc Hepatitis and liver dysfunction with rifampicin therapy for pruritus in primary biliary cirrhosis
    M I Prince
    Centre for Liver Research, University of Newcastle, Newcastle, UK
    Gut 50:436-9. 2002
    ..Rifampicin caused significant hepatitis in 7.3% (95% confidence interval 2.5-19.4%) of patients treated for cholestatic liver disease in our centre...
  81. ncbi Epidemiology and natural history of primary biliary cirrhosis in a US community
    W R Kim
    Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, Minnesota 55905, USA
    Gastroenterology 119:1631-6. 2000
    ..We report the incidence and prevalence of this condition in the general population. We also examined the validity of the Mayo natural history model for PBC among these unselected patients from the community...
  82. ncbi Primary biliary cirrhosis: incidence and predictive factors of cirrhosis development in ursodiol-treated patients
    Christophe Corpechot
    Service d Hepatologie, Hopital Saint Antoine, Paris, France
    Gastroenterology 122:652-8. 2002
    ..This study aimed to assess the incidence of cirrhosis in UDCA-treated patients with PBC and to determine the predictive factors of cirrhosis development under this treatment...
  83. ncbi Characterization of biliary intra-epithelial lymphocytes at different anatomical levels of intrahepatic bile ducts under normal and pathological conditions: numbers of CD4+CD28- intra-epithelial lymphocytes are increased in primary biliary cirrhosis
    Kumiko Isse
    Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan
    Pathol Int 56:17-24. 2006
    ..Altered distribution and phenotypes of bIEL in PBC and hepatolithiasis may reflect their participation in biliary lesions. Increased CD4(+)CD28(-)bIEL in damaged bile ducts of PBC may be related to immune-mediated biliary damage...
  84. pmc Pathogenesis of primary biliary cirrhosis
    David E J Jones
    Liver Research Group, Institute of Cellular Medicine, 4th Floor William Leech Building, The Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, UK
    Gut 56:1615-24. 2007
  85. ncbi Antibodies against homologous microbial caseinolytic proteases P characterise primary biliary cirrhosis
    Dimitrios Petrou Bogdanos
    Immunology Group, Institute of Hepatology, University College London Medical School and Hospitals, Harold Samuel House, 69 75 Chenies Mews, London WC1E 6HX, UK
    J Hepatol 36:14-21. 2002
    ..Since ClpP is highly conserved among bacteria we investigated whether the micro-organisms triggering these antibodies may be other than E. coli...
  86. ncbi Epithelial inflammation is associated with CCL28 production and the recruitment of regulatory T cells expressing CCR10
    Bertus Eksteen
    Liver Research Laboratories, Medical Research Council for Immune Regulation, Institute for Biomedical Research, University of Birmingham, Birmingham B15 2TH, UK
    J Immunol 177:593-603. 2006
    ..We propose that CXCR3 promotes the recruitment of Tregs to inflamed tissues and CCR10 allows them to respond to CCL28 secreted by epithelial cells resulting in the accumulation of CCR10+ Tregs at mucosal surfaces...
  87. pmc Osteoporosis in primary biliary cirrhosis revisited
    J Newton
    Centre for Liver Research, University of Newcastle, Newcastle upon Tyne, UK
    Gut 49:282-7. 2001
    ..Significant improvement at the LS was seen in patients undergoing liver transplantation. CONCLUSIONS: Osteoporosis is not a specific complication of PBC...
  88. ncbi Immunization with a xenobiotic 6-bromohexanoate bovine serum albumin conjugate induces antimitochondrial antibodies
    Patrick S C Leung
    Division of Rheumatology, Allergy and Clinical Immunology, School of Medicine, University of California, Davis, CA 95616, USA
    J Immunol 170:5326-32. 2003
    ....
  89. ncbi Oxidative-stress-related changes in the livers of bile-duct-ligated rats
    Yi Tsau Huang
    Institute of Traditional Medicine, School of Medicine, National Yang Ming University, Taipei, Taiwan, ROC
    J Biomed Sci 10:170-8. 2003
    ..Our results suggest that bile duct ligation in the rat induces mitochondrial dysfunction and biochemical and molecular changes related to oxidative stress in the liver...
  90. ncbi Genetic factors in the pathogenesis of primary biliary cirrhosis
    David E J Jones
    Immunology Research Group, School of Clinical Medical Sciences, University of Newcastle, 4th Floor William Leech Building, Framlington Place, Newcastle upon Tyne NE2 4HH, UK
    Clin Liver Dis 7:841-64. 2003
    ..The possibility of identifying, in the early stages of disease, patients who are at elevated risk for more rapid progression, would have obvious clinical benefit in terms of patient management and therapy...
  91. pmc The 210-kD nuclear envelope polypeptide recognized by human autoantibodies in primary biliary cirrhosis is the major glycoprotein of the nuclear pore
    J C Courvalin
    Centre de Genetique Moleculaire, Centre National de la Recherche Scientifique, Gif sur Yvette, France
    J Clin Invest 86:279-85. 1990
    ..In this study, we show that all of these autoantibodies are directed against a 210-kD integral membrane glycoprotein of the nuclear pore...
  92. ncbi Scavenger cells with gram-positive bacterial lipoteichoic acid infiltrate around the damaged interlobular bile ducts of primary biliary cirrhosis
    K Tsuneyama
    Department of Pathology (II, School of Medicine, Kanazawa University, Japan
    J Hepatol 35:156-63. 2001
    ..Such LTA may be involved in the development and progression of portal tract lesions, particularly bile duct lesions, in PBC...
  93. pmc The X and why of xenobiotics in primary biliary cirrhosis
    Roman Rieger
    Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis, School of Medicine, 451 East Health Sciences Drive, Suite 6510, Davis, CA 95616, USA
    J Autoimmun 28:76-84. 2007
    ..In addition, we will provide a working hypothesis how xenobiotic modification of antigens might occur that ultimately leads to the breaking of self-tolerance and the induction of PBC...
  94. ncbi Medical treatment of primary biliary cirrhosis and primary sclerosing cholangitis
    J Holtmeier
    Medizinische Klinik II, , Theodor-Stern-Kai 7, D-60590 Frankfurt am Main, Germany
    Digestion 64:137-50. 2001
    ..Treatment of extrahepatic manifestations of cholestatic liver disease such as pruritus, fatigue, osteoporosis and steatorrhea can be problematic and time-consuming...
  95. pmc Hepatic IL-17 responses in human and murine primary biliary cirrhosis
    Ruth Y Z Lan
    Division of Rheumatology, Allergy and Clinical Immunology, Genome and Biomedical Sciences Facility, University of California at Davis, 451 East Health Sciences Drive, Suite 6510, Davis, CA 95616, USA
    J Autoimmun 32:43-51. 2009
    ....
  96. ncbi HLA class II alleles, genotypes, haplotypes, and amino acids in primary biliary cirrhosis: a large-scale study
    Peter T Donaldson
    School of Clinical Medical Sciences Hepatology, Faculty of Medical Sciences, University of Newcastle, Newcastle upon Tyne, and Institute of Liver Studies, Kings College Hospital, London, UK
    Hepatology 44:667-74. 2006
    ..042, OR=0.65 in the UK patients; and 10% versus 31%, P=.00092, OR=0.25 in the Italian patients). In conclusion, a complex relationship exists between HLA and PBC, and some genetic associations may be population specific...
  97. ncbi Assessment of biliary fibrosis by transient elastography in patients with PBC and PSC
    Christophe Corpechot
    Service d Hepatologie, Centre national de référence des maladies inflammatoires du foie et des voies biliaires, Hopital Saint Antoine, Paris, France
    Hepatology 43:1118-24. 2006
    ..In conclusion, transient elastography is a simple and reliable noninvasive means for assessing biliary fibrosis. It should be a promising tool to assess antifibrotic therapies in PBC or PSC...
  98. ncbi Primary biliary cirrhosis: report of a focus study group
    Nora V Bergasa
    State University of New York, Downstate Medical Center, Brooklyn, NY 11203, USA
    Hepatology 40:1013-20. 2004
  99. ncbi Human leukocyte antigen class II alleles in Caucasian women with primary biliary cirrhosis
    M E Mullarkey
    Immunogenetics Program, Fred Hutchinson Cancer Research Center, 1100 Fairview Ave N, Seattle, WA 98109, USA
    Tissue Antigens 65:199-205. 2005
    ....
  100. ncbi Isolation and characterization of cDNA encoding a human nuclear antigen predominantly recognized by autoantibodies from patients with primary biliary cirrhosis
    C Szostecki
    Max Planck Institut fur Biochemie, Martinsried, Federal Republic of Germany
    J Immunol 145:4338-47. 1990
    ....
  101. pmc The unfinished business of primary biliary cirrhosis
    Carlo Selmi
    Division of Internal Medicine and Liver Unit, San Paolo Hospital School of Medicine, University of Milan, Italy
    J Hepatol 49:451-60. 2008
    ..However, this research has still not led to successful translation for specific therapy...

Research Grants62

  1. Histamine modulation of biliary proliferation and damage
    Heather L Francis; Fiscal Year: 2013
    ..synthesis may be important in the inhibition of biliary hyperplasia, overexpression of HDC (leading to enhanced histamine release) may be key for stimulating biliary proliferation in ductopenic conditions associated with biliary damage ..
  2. GROWTH REGULATION OF THE INTRAHEPATIC BILIARY TREE
    GIANFRANCO D ALPINI; Fiscal Year: 2010
    ..It is our firm belief that the results of these studies will provide information that will not only allow us to better understand cholangiocyte pathobiology but will also lead to treatments for cholestatic liver diseases. ..
  3. Role of the Renin-Angiotensin System in the Regulation of Biliary Proliferation
    SHANNON STROUD GLASER; Fiscal Year: 2010
    ....
  4. Developing Novel Serological Biomarkers for Autoimmune Liver Diseases
    Xuhang Li; Fiscal Year: 2013
    ....
  5. MECHANISMS AND PATHOGENESIS OF PRIMARY BILIARY CIRRHOSIS
    MERRILL E GERSHWIN; Fiscal Year: 2013
    ..In this application, we will take advantage of that data and define in more precise terms the mechanisms that lead to liver damage in the hopes of developing new therapeutic approaches. ..
  6. dnTGF Beta RII Mice and PBC
    William M Ridgway; Fiscal Year: 2013
    ..We submit that use of our unique murine constructs and our rigorous dissection of the mechanism of autoimmune cholangitis in this model will provide important clues towards successful modulation of human PBC. ..
  7. Mechanisms of xenobiotic-induced biliary inflammation and fibrosis
    James P Luyendyk; Fiscal Year: 2013
    ....
  8. Genomics of Primary Biliary Cirrhosis
    Konstantinos N Lazaridis; Fiscal Year: 2013
    ..Overall, this study will further our understanding of genetic variants and disease mechanisms of PBC that have meaningful clinical impact on individualizing therapy and prognosis. ..
  9. Dysregulated Hypothalamic-pituitary-adrenal Axis During Biliary Hyperplasia
    Sharon Demorrow; Fiscal Year: 2013
    ..This knowledge may play a paramount role in the development of therapeutic strategies for the treatment of cholangiopathies. ..
  10. Regulation of Bile Duct Growth in Bile Duct Ligated Rats
    SHANNON STROUD GLASER; Fiscal Year: 2013
    ..Finally, results of these studies may impact the development of improved therapeutic strategies (targeting AANAT) for the treatment of chronic cholestatic liver diseases. ..
  11. Role of Sensory Neuropeptides in the Regulation of Biliary Function
    Fanyin Meng; Fiscal Year: 2013
    ..This newly acquired fundamental knowledge about regulation of differentiation by sensory neuropeptide-regulated mRNAs and miRNAs in biliary progenitors will advance the general field of cholangiocyte biology. ..
  12. Primary biliary cirrhosis: molecular genetics and microbial pathogenesis
    Jochen Mattner; Fiscal Year: 2013
    ..BENDELAC University of Chicago Consultant Hildeman, David DHILDE CCHMC Consultant University of Pittsburgh ..
  13. XENOBIOTICS AND PRIMARY BILIARY CIRRHOSIS
    MERRILL E GERSHWIN; Fiscal Year: 2013
    ....
  14. Immunogenetic Control of Autoimmune Liver Disease
    William M Ridgway; Fiscal Year: 2013
    ..Understanding early mechanisms of altered biliary immunity will lead to new hypotheses regarding the etiology and treatment of human autoimmune biliary disease. ..
  15. Mechanisms of Nucleocytoplasmic Transport
    Siegfried M Musser; Fiscal Year: 2012
    ....
  16. Novel Proteomic Arrays of In Vitro Expressed Proteins for Autoimmune Disease
    Mark Lim; Fiscal Year: 2009
    ..We propose to extensively optimize and evaluate this new technology during Phase II with the aim of commercializing diagnostic assays for autoimmune diseases. ..
  17. MECHANISMS AND PATHOGENESIS OF PRIMARY BILIARY CIRRHOSIS
    MERRILL E GERSHWIN; Fiscal Year: 2010
    ..We submit that this rigorous dissection of PBC will define pathways that will direct us to potential: mmunotherapeutic agents. ..
  18. The role of CDK5 in cholangiocyte differentiation and morphogenesis
    JUSTIN MICHAEL NUSSBAUM; Fiscal Year: 2012
    ..The goal of this work is in develop a better understanding of cholangiocyte cell biology that will lead to potential therapeutic targets. ..
  19. EPIDEMIOLOGY OF PBC: A GENOME-WIDE ASSOCIATION STUDY
    MERRILL E GERSHWIN; Fiscal Year: 2011
    ..Lay Summary: In this application we hope to determine the genetic basis that leads to a prototypic autoimmune disease, called primary biliary cirrhosis. ..
  20. Single-Molecule Three-Dimensional Snapshots of Nuclear Transport
    Weidong Yang; Fiscal Year: 2010
    ..In this proposal, we employ novel single molecule approaches to further unravel the nuclear transport mechanism. ..
  21. Three-Dimensional Super-Resolution Microscopy Study of Nuclear Transport
    Weidong Yang; Fiscal Year: 2013
    ..Success of this project will significantly change the landscape of research on nuclear transport and also provide new approaches to biomedical imaging, drug development, and medical diagnostics and treatments of these human diseases. ..
  22. URSODIOL-METHOTREXATE FOR PRIMARY BILIARY CIRRHOSIS
    Burton Combes; Fiscal Year: 2003
    ..The safety of each therapeutic regimen is also being determined. ..
  23. GRANULOMA REGULATION THROUGH SOMATOSTATIN RECEPTOR SSTR2
    David Elliott; Fiscal Year: 2000
    ..Our hope is that these discoveries will lead to new therapies for IBD. ..
  24. METHODOLOGIC RESEARCH IN SIMULATING COMPLEX DISEASE
    Mark Roberts; Fiscal Year: 1991
    ..Survival and costs are directly linked, and the timing decision impacts both survival and resources spent on ESLD...
  25. PILOT STUDY OF PIRFENIDONE IN PBC AND PSC
    Keith Lindor; Fiscal Year: 1999
    ..Our ultimate aim is to be able to offer these patients safe and effective therapy for their liver disease, a goal which we have not yet achieved. ..
  26. HEPATIC LESIONS IN MURINE CHRONIC GRAFT-VS-HOST DISEASE
    John Vierling; Fiscal Year: 1991
    ..Clones will be established both by selection of T cells proliferating in response to IL-2 in vitro and by selection of antigen- specific T cells proliferating in response to simulation with isolated bile duct epithelial cells...
  27. XENOBIOTICS AND PRIMARY BILIARY CIRRHOSIS
    MERRILL GERSHWIN; Fiscal Year: 2001
    ..abstract_text> ..
  28. BILIARY CALMODULIN DEPENDENT PROTEIN KINASE II ISOFORMS
    ANN STANSFIELD; Fiscal Year: 2000
    ..These experiments are unique since they will identify and characterize specific CaMKII isoforms involved in regulating Ca2+-dependent Cl- secretion. ..
  29. APOPTOSIS IN HEPATOCYTES
    Harriet Isom; Fiscal Year: 2001
    ..3) To determine how DMSO removal sensitizes hepatocytes to TNFalpha/Fas- induced apoptosis. ..
  30. Immunodeficiencies and Autoimmune Diseases (a Scientific Colloquium)
    Noel Rose; Fiscal Year: 2009
    ..By bringing together geneticists, hematologists, infectious disease specialists, and immunologists together with molecular scientists we intend to challenge and expand the way we presently think about the regulation of immune responses. ..
  31. CLINICAL SIGNIFICANCE OF NOVEL PBC AUTOANTIBODIES
    Donald Bloch; Fiscal Year: 2001
    ..In addition, identification and characterization of novel components of the nuclear body will provide insight into the role of this structure in the pathogenesis of PBC. ..
  32. URSODEOXYCHOLATE-METHOTREXATE FOR BILIARY CIRRHOSIS
    Burton Combes; Fiscal Year: 1992
    ..The mechanism of action is unknown but felt to be related to antiinflammatory-immunosuppressive effects of MTX. The current proposal will explore whether MTX improves the therapeutic effects of UDCA in precirrhotic PBC...
  33. Gene delivery to bile duct epithelial cells in mice
    Vladimir Subbotin; Fiscal Year: 2001
    ..The phase I grant will determine the feasibility of delivering genes into the disease associated cells using non-viral vectors. ..
  34. AUTOANTIGENS AND THE NUCLEAR BODY
    Donald Bloch; Fiscal Year: 2000
    ..4) Determine the clinical significance of autoantibodes directed against components of the NB in patients with PBC. Serum from patients with biopsy-documented PBC will be screened for antibodies directed against the NB. ..
  35. HLA RELATIONSHIPS OF PREGNANCY, CHIMERISM & AUTOIMMUNITY
    J Nelson; Fiscal Year: 1999
    ..The purpose of the proposed studies is to define the role of the maternal- fetal HLA relationship in chimerism resulting from pregnancy and to examine the potential significance of this with respect to the autoimmune disease, SLE. ..
  36. Autoantigen Arrays to Select Vaccines for Model of PBC
    PAUL UTZ; Fiscal Year: 2003
    ..Successful treatment of EAC using plasmids encoding prominent autoantigens may herald an era of customized, antigen- or tissue- specific tolerizing therapy in humans. ..
  37. HEPATIC LESIONS IN MURINE CHRONIC GRAFT VS HOST DISEASE
    John Vierling; Fiscal Year: 2001
    ..New insights into T cell- and cytokine-mediated mechanisms of NSDC immunopathogenesis are needed to develop more effective therapies for PBC and CGVHD. ..
  38. ACQUISITION OF MALDI/TOF MASS SPECTROMETER
    Bruce Hammock; Fiscal Year: 1999
    ..Many of the users NIH funded projects will be facilitated by the capabilities of a high performance MALDI/TOF mass spectrometer. ..
  39. CHRONIC GVH DISEASE IN RAT RADIATION CHIMERAS
    William Beschorner; Fiscal Year: 1990
    ..tt) ..
  40. EPIGENETICS X-LINKED GENES IN PBC: DOES X MARK THE SPOT?
    Carlo Selmi; Fiscal Year: 2007
    ..unreadable] [unreadable] [unreadable]..
  41. dnTGF Beta RII Mice and PBC
    M Gershwin; Fiscal Year: 2009
    ..Our goal is to extend our pilot data to rigorously determine the validity of this model for human PBC and to enable focused future studies directed at mechanisms and potential therapy for PBC. ..
  42. CONSTRUCTION & EVALUATING STATISTICAL PROGNOSTIC SYSTEMS
    Francoise Seillier Moiseiwitsch; Fiscal Year: 1999
    ....
  43. PATHOGENESIS OF CHRONIC GRAFT VERSUS HOST LIVER DISEASE
    Charles Howell; Fiscal Year: 2001
    ..They will perform these experiments by using knockout mice and neutralization of IL12 and INF gamma by treatment with cytokines. ..
  44. Longitudinal Studies of Primary Biliary Cirrhosis
    MARLYN MAYO; Fiscal Year: 2006
    ..In addition, the correlation between each of the putative surrogate markers will be analyzed. The results of this analysis will then be used to design future clinical trials in the field of PBC. [unreadable] [unreadable]..
  45. ROLE OF CD40-CD40 LIGAND IN PRIMARY BILIARY CIRRHOSIS
    MARLYN MAYO; Fiscal Year: 2003
    ..These studies will provide insight into the role of T cells in the pathogenesis of PBC and provide information useful in developing new treatment modalities. ..
  46. 1Treatment of Cholestatic Pruritus With Sertraline
    MARLYN MAYO; Fiscal Year: 2004
    ..These studies will serve as pilot trials. The results of this proposed project will be used to design a larger controlled trial that could be proposed for funding through an R01 mechanism. ..
  47. THE PATHOGENESIS OF AUTOIMMUNITY IN A MURINE MODEL OF PRIMARY BILIARY CIRRHOSIS
    MERRILL E GERSHWIN; Fiscal Year: 2010
    ..abstract_text> ..
  48. BORAGE OIL AND GINKGO BILOBA (EGb 761) IN ASTHMA
    MERRILL GERSHWIN; Fiscal Year: 2004
    ..It is hoped that findings from these studies will evolve relatively non-toxic therapeutic alternatives for attenuating bronchial hyperresponsiveness and inflammation in patients with asthma. ..
  49. Epidemiology and Impact of Hepatitis C in the Community
    W Ray Kim; Fiscal Year: 2007
    ..abstract_text> ..
  50. Regulation of Bile Ductular Proliferation
    JONATHAN DRANOFF; Fiscal Year: 2009
    ..We anticipate that the results of these proposed experiments will lead to novel pharmacologic approaches for the regulation of bile ductular proliferation in biliary cirrhosis. ..
  51. Dissecting the Aggregation of AMA in Families of PBC Patients
    Konstantinos Lazaridis; Fiscal Year: 2008
    ..If successful, our effort will shed light toward better understanding of the genetic predisposition to PBC. [unreadable] [unreadable]..
  52. Oxidative mutagenesis:Iron, Ascorbic Acid and Genes
    Christopher Bowlus; Fiscal Year: 2002
    ..They will use a mouse model of HH that is prone to hepatocellular formation. ..
  53. RETROVIRAL ETIOLOGY OF PRIMARY BILIARY CIRRHOSIS
    Andrew Mason; Fiscal Year: 2002
    ....