Autoantigens of the nuclear pore complex - PubMed (original) (raw)

Review

Autoantigens of the nuclear pore complex

P Enarson et al. J Mol Med (Berl). 2004 Jul.

Abstract

The nuclear envelope (NE) is one of many intracellular targets of the autoimmune response in patients with autoimmune liver disease, systemic lupus erythematosus, and related conditions. In eukaryotic organisms the NE consists of five interconnected regions: an outer nuclear membrane (ONM) that is continuous with the endoplasmic reticulum, an intermembrane or perinuclear space, an inner nuclear membrane (INM) with a unique set of integral membrane proteins, the underlying nuclear lamina, and the pore domains that are regions where the ONM and INM come together. The pore domains are sites of regulated continuity between the cytoplasm and nucleus that are occupied by supramolecular structures, termed nuclear pore complexes (NPCs). Human autoantibodies identified to date bind to specific components in three of the five NE compartments. Autoantigen targets include the lamins A, B, and C of the nuclear lamina, gp210, p62 complex proteins, Nup153, and Tpr within the NPC, and LBR, MAN1, LAP1, and LAP2 that are integral proteins of the INM. Autoantibodies to these NE targets have been shown to be correlated with various autoimmune diseases such as primary biliary cirrhosis, other autoimmune liver diseases and systemic rheumatic diseases. Now that the proteome of the NE is more clearly defined, other autoantibodies to components in this cell compartment are likely to be defined.

PubMed Disclaimer

Similar articles

Cited by

References

    1. Mol Biol Cell. 2003 May;14(5):1923-40 - PubMed
    1. Arthritis Rheum. 2002 Apr;46(4):885-93 - PubMed
    1. Proc Natl Acad Sci U S A. 1983 Jul;80(14):4374-8 - PubMed
    1. EMBO J. 1990 May;9(5):1495-502 - PubMed
    1. Clin Immunol Immunopathol. 1995 Jan;74(1):89-99 - PubMed

Publication types

MeSH terms

Substances

LinkOut - more resources