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Cgnz1 allele confers kidney resistance to damage preventing progression of immune complex–mediated acute lupus glomerulonephritis

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      The mechanisms regulating acute and chronic glomerulonephritis are dependent on different genetic mechanisms, where the Cgnz1 allele confers kidney protection in immune complex–mediated proliferative lupus nephritis.


      Cgnz1 and Agnz1 on the distal region of mouse chromosome 1 are associated with chronic glomerulonephritis (cGN) and acute GN (aGN). NZM2328.Lc1R27 (R27) was generated by introgressing a C57L/J region where Cgnz1 is located to NZM2328. R27 female mice developed aGN mediated by immune complex (IC) deposition and complement activation without progression to cGN with severe proteinuria. End stage renal disease (ESRD) was not seen in R27 mice as old as 15 mo. Thus, aGN and cGN are under separate genetic control, and IC-mediated proliferative GN need not progress to cGN and ESRD. NZM2328 and R27 female mice have comparable immune and inflammatory parameters. In contrast to NZM2328, R27 mice were resistant to sheep anti–mouse GBM serum-induced nephritis, supporting the hypothesis that aGN is mediated by autoimmunity and resistance to the development of cGN is mediated by end organ resistance to damage. Thus, autoimmunity should be considered distinct from end organ damage. The Cgnz1 region has been mapped to a 1.34 MB region with 45 genes. Nine candidate genes were identified. Clinical relevance of these observations is supported by case studies. Clinical implications and the significance to human lupus and other diseases are presented.

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      Most cited references 34

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      Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus.

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        Updating the American college of rheumatology revised criteria for the classification of systemic lupus erythematosus

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          The 1982 revised criteria for the classification of systemic lupus erythematosus.

          The 1971 preliminary criteria for the classification of systemic lupus erythematosus (SLE) were revised and updated to incorporate new immunologic knowledge and improve disease classification. The 1982 revised criteria include fluorescence antinuclear antibody and antibody to native DNA and Sm antigen. Some criteria involving the same organ systems were aggregated into single criteria. Raynaud's phenomenon and alopecia were not included in the 1982 revised criteria because of low sensitivity and specificity. The new criteria were 96% sensitive and 96% specific when tested with SLE and control patient data gathered from 18 participating clinics. When compared with the 1971 criteria, the 1982 revised criteria showed gains in sensitivity and specificity.

            Author and article information

            [1 ]Division of Rheumatology and Immunology and [2 ]Center of Immunity, Inflammation and Regenerative Medicine, Department of Medicine ; [3 ]Department of Microbiology, Immunology and Cancer Biology ; [4 ]Division of Nephrology, Department of Medicine ; [5 ]Department of Pharmacology ; [6 ]Department of Pathology ; and [7 ]Department of Psychiatry and Neurobehavioral Sciences, University of Virginia School of Medicine, Charlottesville, VA 22908
            Author notes
            CORRESPONDENCE Shu Man Fu: sf2e@

            Y. Ge’s present address is Genetics Institute, University of Florida, Gainesville, FL 32610.

            C. Jiang’s present address is Molecular Immunology Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD 20814.

            J Exp Med
            J. Exp. Med
            The Journal of Experimental Medicine
            The Rockefeller University Press
            21 October 2013
            : 210
            : 11
            : 2387-2401
            24101379 3804943 20130731 10.1084/jem.20130731
            © 2013 Ge et al.

            This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 3.0 Unported license, as described at




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