Primary biliary cirrhosis and the nuclear pore complex

Carolina Duarte-Rey, Dimitrios Bogdanos, Chen Yen Yang, Krista Roberts, Patrick S C Leung, Juan Manuel Anaya, Howard J. Worman, M. Eric Gershwin

Resultado de la investigación: Contribución a RevistaRevisión Literaria

19 Citas (Scopus)

Resumen

Experimental models of autoimmune diseases have led to the conclusion that an immune response to nuclear antigens is a sentinel marker for loss of tolerance and potential tissue damage. Various proteins are targets of antinuclear antibodies in a variety of autoimmune diseases, ranging from systemic rheumatologic disorders to diseases affecting specific organs such as the liver. Autoantibodies against specific nuclear constituents have also been used as probes to understand the structure and the function of the targeted components and their relevance to disease pathogenesis. Approximately a quarter of patients with primary biliary cirrhosis (PBC) have antibodies targeting proteins of the nuclear pore complex (NPC), a multi-protein structure that mediates molecular transport across the nuclear envelope. Autoantibodies against the integral membrane glycoprotein gp210 and nucleoporin p62 appear to be highly specific for PBC, an autoimmune disease characterized by progressive destruction of intrahepatic biliary epithelial cells. This review discusses the diagnostic and clinical relevance of anti-NPC antibodies in PBC and the possibility that this autoimmune response may arise as a result of molecular mimicry. © 2012 Elsevier B.V.
Idioma originalEnglish (US)
Páginas (desde-hasta)898-902
Número de páginas5
PublicaciónAutoimmunity Reviews
DOI
EstadoPublished - oct 1 2012

Huella dactilar

Nuclear Pore
Biliary Liver Cirrhosis
Autoimmune Diseases
Autoantibodies
Nuclear Pore Complex Proteins
Molecular Mimicry
Nuclear Antigens
Antibodies
Antinuclear Antibodies
Membrane Glycoproteins
Nuclear Envelope
Molecular Structure
Autoimmunity
Proteins
Theoretical Models
Epithelial Cells
Liver

Citar esto

Duarte-Rey, C., Bogdanos, D., Yang, C. Y., Roberts, K., Leung, P. S. C., Anaya, J. M., ... Gershwin, M. E. (2012). Primary biliary cirrhosis and the nuclear pore complex. Autoimmunity Reviews, 898-902. https://doi.org/10.1016/j.autrev.2012.03.005
Duarte-Rey, Carolina ; Bogdanos, Dimitrios ; Yang, Chen Yen ; Roberts, Krista ; Leung, Patrick S C ; Anaya, Juan Manuel ; Worman, Howard J. ; Gershwin, M. Eric. / Primary biliary cirrhosis and the nuclear pore complex. En: Autoimmunity Reviews. 2012 ; pp. 898-902.
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Duarte-Rey, C, Bogdanos, D, Yang, CY, Roberts, K, Leung, PSC, Anaya, JM, Worman, HJ & Gershwin, ME 2012, 'Primary biliary cirrhosis and the nuclear pore complex', Autoimmunity Reviews, pp. 898-902. https://doi.org/10.1016/j.autrev.2012.03.005

Primary biliary cirrhosis and the nuclear pore complex. / Duarte-Rey, Carolina; Bogdanos, Dimitrios; Yang, Chen Yen; Roberts, Krista; Leung, Patrick S C; Anaya, Juan Manuel; Worman, Howard J.; Gershwin, M. Eric.

En: Autoimmunity Reviews, 01.10.2012, p. 898-902.

Resultado de la investigación: Contribución a RevistaRevisión Literaria

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Duarte-Rey C, Bogdanos D, Yang CY, Roberts K, Leung PSC, Anaya JM y otros. Primary biliary cirrhosis and the nuclear pore complex. Autoimmunity Reviews. 2012 oct 1;898-902. https://doi.org/10.1016/j.autrev.2012.03.005