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| 1 | 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 |
| 2 | Use of platelet-rich plasma in the care of sports injuries:our experience with ultrasoundguided injection显示文摘 | Bernuzzi G Petraglia P Pedrini M F | 2014 | Trasfusione del sangue2014，12，1： | 1 |
| 3 | Identification of serum and tissue micro-RNA expression profiles in different stages of inflammatory boweldisease显示文摘 | Iborra M Bernuzzi F Correale C | 2013 | Clin Exp Immunol2013，173，2： | 1 |
| 4 | MicroRNAs in autoim- munity and inflammatory bowel disease: crucial regulators in im- mune response显示文摘 | Iborra M Bernuzzi F Invernizzi P | 2012 | Autoimmun Rev2012，11，5： | 1 |
| 5 | E- valuation of factors influencing ampoule secondary particulate contamination 显示文摘 | Bernuzzi M Raggi P Montanari L | 1991 | Boll Chim Farm1991，130，8： | 1 |
| 6 | In vitro efficacy of nikkomycin Z against the human isolate of the microsporidian species Encephalitozoon hellem显示文摘 | Bigliardi E Bernuzzi A M Corona S | 2000 | Antimicrob Agents Chemother2000，44，： | 1 |
| 7 | MicroRNAs inautoimmunity and inflammatory bowel disease:Crucialregulators in immune response显示文摘 | Iborra M Bernuzzi F Invernizzi P | 2012 | Autoimmun Rev2012，11，5： | 1 |
| 8 | Evaluation of factors influencing ampoule secondary partculate contamination显示文摘 | Bernuzzi M Raggi P | 1991 | Boll Chin Farm1991，130，8： | 1 |
| 9 | Evaluation of factors influencing ampoule secondary particulate contamination显示文摘 | BERNUZZI M RAGGI P | 1991 | Boll Chin Farm1991，130，8： | 1 |
| 10 | In vitro efficacy of nikkomycin Z against the human isolate of the microsporidian species Encephalitozoon hellem显示文摘 | BIGLIARDI E BERNUZZI A M CORONA S | 2000 | Antimicrobiai Agents and Chemotherap2000，44，11： | 1 |
| 11 | In vitro efficacy of nikkomyein Z against the human isolate of the microsporidian species Encephalitozoon hellern显示文摘 | BIGIAARDI E BERNUZZI A M CORONA S | 2000 | Antimicrobial Agents and Chemotherap2000，44，11： | 1 |
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