AC-12 - Punctate Nuclear Envelope
Descrição
Nuclear envelope reveals a punctate staining in interphase cells, with accentuation of fluorescence at the points where adjacent cells touch each other. No staining of the metaphase and anaphase chromatin plates. e.g. anti-gp210.
Associação Antigênica
nuclear pore complex proteins
Doenças Associadas
PBC
Relevâncias Clínicas - Primeiro nível
Found in patients with PBC, as well as patients with other autoimmune liver diseases and SARD.
If PBC is clinically suspected, it is recommended to perform a follow-up test for anti-gp210 antibodies; the antigen is included in disease specific immunoassays (ie, extended liver profile).
Other antigens recognized include p62 nucleoporin, LBR, and Tpr; specific immunoassays for these autoantibodies are currently not commercially available.
Relevâncias Clínicas - Segundo nível
Anti-p62 nucleoporin antibodies have been described in PBC and SLE.
Anti-LBR antibodies have been described in PBC.
Anti-Tpr antibodies have been described in PBC, autoimmune liver disease, SLE, SSc and SjS.
Most reports describe autoantibodies directly binding to specific antigens (i.e., antigen-specific immunoassays) and do not actually show clear correlations with the AC-12 pattern as such; specific immunoassays for these autoantibodies are currently not commercially available.
Referências
Bandin O, Courvalin JC, Poupon R, et al. Specificity and sensitivity of gp210 autoantibodies detected using an enzyme-linked immunosorbent assay and a synthetic polypeptide in the diagnosis of primary biliary cirrhosis. Hepatology 1996;23:1020–4.
Courvalin JC, Lassoued K, Bartnik E, et al. The 210-kD nuclear envelope polypeptide recognized by human autoantibodies in primary biliary cirrhosis is the major glycoprotein of the nuclear pore. J Clin Invest 1990;86:279–85.
Courvalin JC, Lassoued K, Worman HJ, et al. Identification and characterization of autoantibodies against the nuclear envelope lamin B receptor from patients with primary biliary cirrhosis. J Exp Med 1990;172:961–7.
Kraemer DM, Tony HP. Nuclear pore protein p62 autoantibodies in systemic lupus erythematosus. Open Rheumatol J 2010;4:24–7.
Miyachi K, Hankins RW, Matsushima H, et al. Profile and clinical significance of anti-nuclear envelope antibodies found in patients with primary biliary cirrhosis: a multicenter study. J Autoimmun 2003;20:247–54.
Nickowitz RE, Worman HJ. Autoantibodies from patients with primary biliary cirrhosis recognize a restricted region within the cytoplasmic tail of nuclear pore membrane glycoprotein gp210. J Exp Med 1993;178:2237–42.
Ou Y, Enarson P, Rattner JB, et al. The nuclear pore complex protein TPR is a common autoantigen in sera that demonstrate nuclear envelope staining by indirect immunofluorescence. Clin Exp Immunol 2004;136:379–87.
Wesierska-Gadek J, Klima A, Komina O, et al. Characterization of autoantibodies against components of the nuclear pore complexes: high frequency of anti-p62 nucleoporin antibodies. Ann N Y Acad Sci 2007;1109:519–30.