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Case Reports
. 1998 Mar;111(3):527-31.
doi: 10.1046/j.1365-2249.1998.00523.x.

A 75-kD autoantigen recognized by sera from patients with X-linked autoimmune enteropathy associated with nephropathy

I Kobayashi  1 , K Imamura, M Yamada, M Okano, A Yara, S Ikema, N Ishikawa
Affiliations
Case Reports

A 75-kD autoantigen recognized by sera from patients with X-linked autoimmune enteropathy associated with nephropathy

I Kobayashi et al. Clin Exp Immunol. 1998 Mar.

Abstract

Autoimmune enteropathy (AIE) is a rare disorder characterized by intractable diarrhoea and antienterocyte autoantibody. In this study, we detected a 75-kD autoantigen which distributed through the whole intestine and the kidney, as assessed by Western blot analysis using sera from two unrelated cases of AIE associated with nephropathy. Our results suggest that the detection of the autoantibody against the 75-kD antigen has a diagnostic value in AIE and that the autoimmune reaction against the 75-kD antigen may be implicated in the development of intestinal and renal tissue damage in this rare disorder.

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Figures

Fig. 1
Fig. 1
Autoantibody against the 75-kD antigen is disease-specific. Arrow: 75-kD protein was reactive with serum from cases 1 and 2 (lanes 1 and 2, respectively), but not with sera from a variety of diseases (lanes 3–10; systemic lupus erythematosus, ulcerative colitis, Sjögren's syndrome, chronic active Epstein–Barr virus infection, bronchial asthma, dermatomyositis, juvenile rheumatoid arthritis and common variable immunodeficiency, respectively), normal control (lanes 11 and 12) or γ-globulin (lane 13).
Fig. 2
Fig. 2
Chronological changes in reactivities of sera from case 1 with 75-kD antigen. Sera were obtained at admission (lane 1), remission (lane 2) and relapse (lane 3).
Fig. 3
Fig. 3
Tissue distribution of the 75-kD antigen. The reactivities of sera from case 1 (a) and case 2 (b) with the lysates of the indicated tissues were tested by Western blot analysis.
See this image and copyright information in PMC

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