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| 1 | Experimental evidence on the immunopathogenesis of primary biliary cirrhosis显示文摘Primary biliary cirrhosis(PBC)is a chronic cholestatic liver disease for which an autoimmune pathogenesis is supported by clinical and experimental data,including the presence of autoantibodies and autoreactive T cells.The etiology remains to be determined,yet data suggest that both a susceptible genetic background and unknown environmental factors determine disease onset.Multiple infectious and chemical candidates have been proposed to trigger the disease in a genetically susceptible host,mostly by molecular mimicry.Most recently,several murine models have been reported,including genetically determined models as well as models induced by immunization with xenobiotics and bacteria. | Carlo Selmi Francesca Meda Anaid Kasangian Pietro Invernizzi Zhigang Tian Zhexiong Lian Mauro Podda M Eric Gershwin | 2010 | Cellular & Molecular Immunology2010，7，1： | 14 |
| 2 | Etiopathogenesis of primary biliary cirrhosis显示文摘Primary biliary cirrhosis(PBC) is an autoimmune disease of the liver characterized by progressive bile duct destruction eventually leading to cirrhosis and liver failure.The serological hallmark of the disease is the presence of circulating antimitochondrial antibodies(AMA).These reflect the presence of autoreactive T and B cells to the culprit antigens,the E2 subunits of mitochondrial 2-oxo-acid dehydrogenase enzymes,chiefly pyruvate dehydrogenase(PDC-E2).The disease results from a combination of genetic and environmental risk factors.Genetic predisposition is indicated by the higher familial incidence of the disease particularly among siblings and the high concordance rate among monozygotic twins.Environmental triggering events appear crucial to disrupt a pre-existing unstable immune tolerance of genetic origin allowing,after a long latency,the emergence of clinical disease.Initiating mimetopes of the vulnerable epitope of the PDC-E2 autoantigen can be derived from microbes that utilize the PDC enzyme or,alternatively,environmental xenobiotics/chemical compounds that modify the structure of native proteins to make them immunogenic.A further alternative as a source of antigen is PDC-E2 derived from apoptotic cells.In the effector phase the biliary ductular cell,by reason of itsproclivity to express the antigen PDC-E2 in the course of apoptosis,undergoes a multilineage immune attack comprised of CD4+ and CD8+ T cells and antibody.In this article,we critically review the available evidence on etiopathogenesis of PBC and present interpretations of complex data,new developments and theories,and nominate directions for future research. | Ana Lleo Pietro Invernizzi Ian R Mackay Harry Prince Ren-Qian Zhong M Eric Gershwin | 2008 | World Journal of Gastroenterology2008，14，21： | 9 |
| 3 | Comprehensive review of autoantibodies in patients with hyper-IgM syndrome显示文摘Hyper-immunoglobulin M syndrome is an X-linked primary immunodeficiency disease caused by mutations in the CD40 ligand gene.The CD40 ligand has been recently highlighted as playing a key role in the pathogenesis of primary biliary cholangitis.In the present study,we assessed an extensive set of serum autoantibodies in a series of well-defined patients with hyper-immunoglobulin M syndrome.Serum,liver-related and liver-not-related autoantibodies IgG,IgM and IgA were tested by ELISA and standard indirect immunofluorescence in HEp-2 cells in 13 Tunisian patients(8 males and 5 females,aged 1–12 years)with hyper-immunoglobulin M syndrome during 1995–2012 and,as controls,21 age-and gender-matched blood donors.The level of IgM antibody against MIT3 was significantly higher in patients than in controls(35.8 vs 10.7,P=0.002).Half of the hyperimmunoglobulin M syndrome patients were found to be anti-MIT3 IgM positive vs none of the controls(Po0.0001).Twenty-three percent of patients were found to be anti-sp100 antibody positive vs only 0.05%of controls.By immunofluorescence,92.3%of patients were MIT3 IgM positive vs none of the controls.In conclusion,the IgM class of anti-MIT3 antibodies was shown to be present by both ELISA and immunofluorescence in most of the patients with hyper-immunoglobulin M syndrome.The presence of the hallmark of primary biliary cholangitis,a disease where the CD40 ligand is a key player,in an immunodeficiency disease caused by mutations in the CD40 ligand gene is very intriguing and opens new scenarios in understanding the immune pathogenesis of primary biliary cholangitis. | Mohamed-Ridha Barbouche Qubo Chen Marco Carbone Imen Ben-Mustapha Zakera Shums Mehdi Trifa Federica Malinverno Francesca Bernuzzi Haiyan Zhang Nourhen Agrebi Gary L Norman Christopher Chang M Eric Gershwin Pietro Invernizzi | 2018 | Cellular & Molecular Immunology2018，15，6： | 2 |
| 4 | The frequency of monosomy X in women with primary biliary cirrhosis 显示文摘 | Invernizzi P Miozzo M Battezatti PM | 2004 | Lancet2004，363，： | 1 |
| 5 | Multiobjective optimization for pricing system security in electricity markets显示文摘 | Milano F Canizares C A Invernizzi M | 2003 | IEEE Transactions on Power Systems2003，18，2： | 1 |
| 6 | Black-start and restoration of a part of the Italian HV network: modeling and simulation of a field test显示文摘 | Delfino B Denegri G B Invernizzi M | 1996 | IEEE Transactions on Power Systems1996，11，3： | 1 |
| 7 | Sarcopenia or muscle modifica- tions in neurologic diseases: a lexical or patophysiological difference 显示文摘 | Carda S Cisari C Invernizzi M | 2013 | EurJPhys Rehabil Med2013，49，1： | 1 |
| 8 | Cryoglobulinaemias:a multi-center study of the early clinical and laboratory manifestations of primary and secondary disease显示文摘 | Monti G Galli M Invernizzi F | 1995 | Q J Med1995，88，： | 1 |
| 9 | Interpreting serological tests in diagnosing autoimmune liver diseases显示文摘 | Invernizzi P Lleo A Podda M | 2007 | Semin Liver Dis2007，27，： | 1 |
| 10 | Osteoporosis in Park-inson's disease显示文摘 | Invernizzi M Carda S Viscontini GS | 2009 | Parkinsonism Relat Disord2009，15，5： | 1 |
| 11 | Black-start Andrestoration of a Part of the Italian HV Network: Modeling and Simulation of a Field Test显示文摘 | Delfino B Denegri G B Invernizzi M | 1996 | IEEE Trans on Power Systems1996，11，3： | 1 |
| 12 | Symptomatic ulcerative carotid plaque显示文摘 | Mumoli N Cei M Invernizzi C | 2008 | Circulation2008，118，24： | 1 |
| 13 | for pricing system security in electricity markets显示文摘 | Milano F Canizares C Multiobjective optimization A Invernizzi M | 2003 | IEEE Transactions on Power Systems2003，18，2： | 1 |
| 14 | Primary biliary cirrhosis in monozygotic and dizygotic twins:genetics,epigeneties,and environment显示文摘 | Selmi C Mayo M J Bach N Ishibashi H Invernizzi P Gish R G | 2004 | Gastroenterology2004，127，： | 1 |
| 15 | The value of add- ing mirror therapy for upper limb motor recovery of sub- acute stroke patients: a randomized controlled trial显示文摘 | Invernizzi M Negrini S Carda S | 2013 | Eur J Phys Rehabil Med2013，49，3： | 1 |
| 16 | Black-start and restoration of a part of the Italian HV network:modeling and simulation of a field test显示文摘 | Delfino B Denegri G B Invernizzi M | | 0，，03： | 1 |
| 17 | Ectopic expression of pRb2/p130 suppresses the tumorigenicity of the cerbB - 2 overexpressing SKOV3 tumor cell line 显示文摘 | PUPA S M HOWARD C M INVERNIZZI A M | 1999 | Oncogene1999，18，3： | 1 |
| 18 | Osteoporosis in Park - iason ~ disease 显示文摘 | INVERNIZZI M CARDA S VISCONTINI G S | 2009 | Parkinsonism Relat Disord2009，15，5： | 1 |
| 19 | The prognosis and management of inactive HBV carriers 显示文摘 | Invernizzi F Vigan6 M Grossi G | 2016 | Liver Int2016，361，： | 1 |
| 20 | Heavy metal pollution in topsoils near a cement plant:the role of organic matter and distance to the source to predict total and HCl-extracted heavy metal concentrations 显示文摘 | Bermudez G M A Moreno M Invernizzi R | 2010 | Chemosphere2010，78，4： | 1 |
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