The peculiar autoimmunity of primary biliary cirrhosis

@article{Mackay2000ThePA,
  title={The peculiar autoimmunity of primary biliary cirrhosis},
  author={Ian R. Mackay and Senga Whittingham and Shahnaz Fida and Mark A. Myers and Nobuhiro Ikuno and Merrill Eric Gershwin},
  journal={Immunological Reviews},
  year={2000},
  volume={174}
}
Summary: Autoantibodies to mitochondria (AMA, anti‐M2) are a serologic hallmark of primary biliary cirrhosis (PBC). These react with three structurally and functionally related multienzymic complexes, the 2‐oxoacid dehydrogenase complexes, but chiefly with the E2 subunit of pyruvate dehydrogenase complex (PDC‐E2). Their very close (95%) and specific association with PBC underpins the autoimmune concept of pathogenesis of that disease, notwithstanding several non‐congruent features. Detailed… 
Autoimmunity and primary biliary cirrhosis.
  • I. Mackay
  • Biology, Medicine
    Bailliere's best practice & research. Clinical gastroenterology
  • 2000
TLDR
An accumulation uniquely in PBC of PDC-E2-like material at the plasma membrance of biliary epithelial cells (BECs) provides a credible 'tissue-specific' target for an autoimmune attack by T and B lymphocytes at the site of the actual pathology.
Investigation of immune complexes formed by mitochondrial antigens containing a new lipoylated site in sera of primary biliary cholangitis patients
TLDR
It is hypothesized that AMAs and their antigens formed ICs in PBC sera, and sera of PBC and four autoimmune diseases are analyzed using immune complexome analysis, in which ICs are separated from serum and are identified by nano‐liquid chromatography‐tandem mass spectrometry.
Etiopathogenesis of primary biliary cirrhosis: an overview of recent developments
TLDR
Key advances in the field of primary biliary cirrhosis research are summarized as the true complexities of the disease process begin to be unraveled.
The causes of primary biliary cirrhosis: Convenient and inconvenient truths
TLDR
It is emphasized that the potential initiator of PBC includes inter alia particular environmental xenobiotics; pathogenesis is aided and abetted by genetic weaknesses in mechanisms of immune regulation; and subsequent multilineage immunopathology impacts upon uniquely susceptible BECs to culminate clinically in the chronic autoimmune cholangiolitis of P BC.
Molecular diagnostics of primary biliary cirrhosis.
TLDR
Critical analysis of all published data regarding PBC serology between 1985 and 2007 was performed in order to suggest a diagnostic algorithm for the serological diagnosis of PBC, and molecular-based assays have been developed for the detection of P BC-specific ANA.
Antimitochondrial Antibodies and Reactivity to N. Aromaticivorans Proteins in Icelandic Patients with Primary Biliary Cirrhosis and Their Relatives
TLDR
Interestingly, despite the homogenous genetic background, the group of Icelandic patients with PBC was heterogeneous in their AMA reactive patterns and also reacted with N. aromaticivorans proteins.
Etiopathogenesis of primary biliary cirrhosis.
TLDR
The available evidence on etiopathogenesis of PBC is critically reviewed and interpretations of complex data, new developments and theories, and nominate directions for future research are presented.
The immunobiology and pathophysiology of primary biliary cirrhosis.
TLDR
A better understanding of the mechanism for selective biliary cell destruction is resulted and genome-wide association studies suggest an important role for the IL-12 pathway in disease susceptibility.
Antimitochondrial and other autoantibodies.
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TLDR
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