• We are sorry, but NCBI web applications do not support your browser and may not function properly. More information
Logo of gutGutView this articleSubmit a manuscriptReceive email alertsContact usBMJ
Gut. May 1998; 42(5): 753–756.
PMCID: PMC1727116

Autoantibodies against subunits of pyruvate dehydrogenase and citrate synthase in a case of paediatric biliary cirrhosis


In a newborn girl with a history of connatal liver damage, histological examination of a liver biopsy sample taken during the seventh week of life revealed incipient destruction of bile ducts. Very high titres of antimitochondrial antibodies were later detected in the plasma. As the hepatic injury tended towards fibrosis, the histological diagnosis became primary biliary cirrhosis. Autoantibodies against E, E2, and E3 subunits and protein X component of pyruvate dehydrogenase complex, and against citrate synthase were detected on western immunoblotting in a 1 in 1000 dilution of the patient's serum. The patient died of her illness at 11 years of age. In liver specimens obtained at autopsy human immunoglobulin deposition was detected on the surface of almost all hepatic cells by immunohistology. As there is a physical and functional interaction between pyruvate dehydrogenase and citrate synthase within the mitochondria, the presence of autoantibodies against certain proteins in the patient suggests that in this form of the disease the molecular recognition and then the autoimmunisation process could be directed against a mitochondrial enzyme cluster containing both pyruvate dehydrogenase and citrate synthase.

Keywords: primary biliary cirrhosis; pyruvate dehydrogenase; citrate synthase

Full Text

The Full Text of this article is available as a PDF (193K).

Figures and Tables

Figure 1
Autoantibodies against PDC and CS. Lane A, from the top: E2 (apparent molecular weight 70-74 kDa), protein X (50-56 kDa), E3 (55 kDa), and E1α (41 kDa) subunits of PDC. Lane B: western blotting for CS (50 kDa) showed anticitrate synthase antibody ...
Figure 2
(A) In needle biopsy samples signs of biliary cirrhosis were prominent at six years of age (haematoxylin-eosin staining). (B) Following immunostaining of the liver there was intensive reaction with antihuman immunoglobulin predominantly on the surface ...

Articles from Gut are provided here courtesy of BMJ Group


Related citations in PubMed

See reviews...See all...

Cited by other articles in PMC

See all...


  • MedGen
    Related information in MedGen
  • PubMed
    PubMed citations for these articles
  • Substance
    PubChem Substance links

Recent Activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...