Anti-liver cytosolic antigen type 1 (LC1) antibodies in childhood autoimmune liver disease

Hepatology. 1995 Jan;21(1):58-62.


Antibodies to liver cytosol antigen type 1 (anti-LC1), which recognize a 60-kd peptide contained in the liver cytosolic fraction, have been reported to define a subset of autoimmune hepatitis (AIH) either negative for other autoantibodies or positive for anti-liver kidney microsomal antibody type 1 (LKM-1) and to be best detected in immunodiffusion. To analyze the prevalence of anti-LC1 in childhood liver disease, we have tested the sera of 95 patients using immunoblot, indirect immunofluorescence, and immunodiffusion. Fifteen children had smooth muscle antibody (SMA) and/or anti-nuclear antibody (ANA)-positive AIH, 13 had anti-LKM-1-positive AIH, 14 had autoimmune sclerosing cholangitis (ASC) (all SMA and/or ANA positive), and 53 had non-autoimmune liver disease (10 had alpha 1-anti-trypsin deficiency [alpha 1-ATD], 11 had Wilson's disease [WD], 14 had Alagille's syndrome, and 18 had chronic hepatitis B virus [HBV] infection). Twenty healthy children were studied as controls. Anti-LC1 positivity in immunodiffusion and strong reactivity in immunoblot were found in 4 LKM-1- and 2 SMA/ANA-positive patients with AIH and in 1 patient with ASC, but in none of the patients with other liver diseases nor in controls. A weak 60-kd band was detected by immunoblot in 6 more patients with AIH (2 were LKM-1- and 4 were SMA/ANA-positive) and 6 patients with ASC, all anti-LC1-negative by immunofluorescence and immunodiffusion. No distinct clinical features characterized the anti-LC1-positive patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Animals
  • Autoantibodies / analysis*
  • Autoantigens / immunology*
  • Autoimmune Diseases / immunology*
  • Child
  • Child, Preschool
  • Fluorescent Antibody Technique
  • Humans
  • Immunoblotting
  • Immunodiffusion
  • Infant
  • Liver Diseases / immunology*
  • Male
  • Rabbits
  • Rats
  • Rats, Wistar
  • Species Specificity


  • Autoantibodies
  • Autoantigens
  • liver cytosol antigen type 1, human
  • liver cytosol antigen type 1, rat