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

Clin Exp Immunol. 1998 Mar;111(3):527-31. doi: 10.1046/j.1365-2249.1998.00523.x.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antigens / blood
  • Autoantigens / analysis*
  • Autoantigens / blood
  • Autoimmune Diseases / blood*
  • Autoimmune Diseases / genetics
  • Autoimmune Diseases / immunology
  • Blotting, Western
  • Child
  • Genetic Linkage
  • Humans
  • Infant
  • Intestinal Diseases / blood*
  • Intestinal Diseases / genetics
  • Intestinal Diseases / immunology
  • Intestines / chemistry
  • Kidney / chemistry
  • Kidney Diseases / blood*
  • Kidney Diseases / genetics
  • Kidney Diseases / immunology
  • Male
  • X Chromosome


  • Antigens
  • Autoantigens