Antimitochondrial antibodies in primary biliary cirrhosis

Patrick S Leung, Ross L. Coppel, Aftab Ansari, Santiago Munoz, M. Eric Gershwin

Research output: Contribution to journalArticle

104 Citations (Scopus)

Abstract

In the last decade, the cloning and biochemical identification of mitochondrial autoantigens in primary biliary cirrhosis (PBC) as members of the 2-oxoacid dehydrogenase complex has greatly advanced the detection of antimitochondrial antibodies (AMA) and the understanding of the immunobiology of the disease. Here, we discuss the methods of detecting AMA and its isotypes, methods of epitope mapping, and using these methods in PBC liver immunohistochemistry and Ig gene usage. Increasing evidence, including the specific association of AMA with PBC, the unique similar but noncross- reactive conformational epitope of the lipoyl domains of the mitochondrial autoantigens, the specific binding of anti-PDC-E2 monoclonal antibodies and human combinatorial antibodies derived from PBC patients to the apical area of bile duct epithelial cells in PBC livers, and Ig gene usage of AMA, suggests that AMA is not an epiphenomenon of the disease but plays a significant role in the pathogenesis of PBC.

Original languageEnglish (US)
Pages (from-to)61-69
Number of pages9
JournalSeminars in Liver Disease
Volume17
Issue number1
StatePublished - Feb 1997

Fingerprint

Biliary Liver Cirrhosis
Antibodies
Immunoglobulin Genes
Autoantigens
Keto Acids
Epitope Mapping
Liver
Bile Ducts
Organism Cloning
Epitopes
Oxidoreductases
Epithelial Cells
Immunohistochemistry
Monoclonal Antibodies

Keywords

  • 2-oxo-acid dehydrogenase complex
  • bile duct epithelium
  • cross-reactive molecule
  • epitope
  • Ig gene
  • PDC- E2

ASJC Scopus subject areas

  • Hepatology

Cite this

Antimitochondrial antibodies in primary biliary cirrhosis. / Leung, Patrick S; Coppel, Ross L.; Ansari, Aftab; Munoz, Santiago; Gershwin, M. Eric.

In: Seminars in Liver Disease, Vol. 17, No. 1, 02.1997, p. 61-69.

Research output: Contribution to journalArticle

Leung, PS, Coppel, RL, Ansari, A, Munoz, S & Gershwin, ME 1997, 'Antimitochondrial antibodies in primary biliary cirrhosis', Seminars in Liver Disease, vol. 17, no. 1, pp. 61-69.
Leung, Patrick S ; Coppel, Ross L. ; Ansari, Aftab ; Munoz, Santiago ; Gershwin, M. Eric. / Antimitochondrial antibodies in primary biliary cirrhosis. In: Seminars in Liver Disease. 1997 ; Vol. 17, No. 1. pp. 61-69.
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