Anti-glycoprotein-210 antibodies

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Nucleoporin 210kDa
Autoantigen gene NUP210
Affected organ(s) Bile Duct
Primary biliary cirrhosis
HLA associations DR2 (weak)

Anti-glycoprotein-210 antibodies (AGPA, anti-gp210, anti-nup210, anti-np210) are directed at gp210[1] and are found within primary biliary cirrhosis (PBC) patients in high frequency. AGPA recognize the cytoplasmic-oriented carboxyl terminus (tail) of the protein.[2] While AGPA is found as a prognostic marker in only a minority of PBC patients, those that did had higher mortality and were predicted a poor outcome.[3] In addition, patients that responded to ursodeoxycholic acid (UDCA) therapy and, therefore, had AGPA reductions failed to develop end-stage liver disease relative to untreated cohort with anti-gp210 Ab.[4] PBC patients with potentially destructive AGPA have increased expression of Nup210 in the bile duct, a potential immune tolerance-escaping factor.[5]

Anti-mitochondrial, anti-centromere[6] and anti-p62 antibodies are also found in (PBC). While patients with AGPA progress toward end-stage liver failure, patients with anti-centromere antibodies often progress toward portal hypertension, further indicating a specific role of the AGPA in PBC.


The glycoprotein gp210 is commonly used in the literature. The gene, NUP210, encodes the nuclear pore (nuclear porin) glycoprotein-210 that is a major component of the human nuclear pore complex.


  1. ^ Courvalin JC, Lassoued K, Worman HJ, Blobel G (1990). "Identification and characterization of autoantibodies against the nuclear envelope lamin B receptor from patients with primary biliary cirrhosis". J. Exp. Med. 172 (3): 961–7. doi:10.1084/jem.172.3.961. PMC 2188537. PMID 2167346. 
  2. ^ Nickowitz RE, Worman HJ (1993). "Autoantibodies from patients with primary biliary cirrhosis recognize a restricted region within the cytoplasmic tail of nuclear pore membrane glycoprotein Gp210". J. Exp. Med. 178 (6): 2237–42. doi:10.1084/jem.178.6.2237. PMC 2191303. PMID 7504063. 
  3. ^ Itoh S, Ichida T, Yoshida T, et al. (1998). "Autoantibodies against a 210 kDa glycoprotein of the nuclear pore complex as a prognostic marker in patients with primary biliary cirrhosis". J. Gastroenterol. Hepatol. 13 (3): 257–65. doi:10.1111/j.1440-1746.1998.01553.x. PMID 9570238. 
  4. ^ Nakamura M, Shimizu-Yoshida Y, Takii Y, et al. (2005). "Antibody titer to gp210-C terminal peptide as a clinical parameter for monitoring primary biliary cirrhosis". J. Hepatol. 42 (3): 386–92. doi:10.1016/j.jhep.2004.11.016. PMID 15710222. 
  5. ^ Nakamura M, Takii Y, Ito M, et al. (2006). "Increased expression of nuclear envelope gp210 antigen in small bile ducts in primary biliary cirrhosis". J. Autoimmun. 26 (2): 138–45. doi:10.1016/j.jaut.2005.10.007. PMID 16337775. 
  6. ^ Nakamura M, Kondo H, Mori T, et al. (2007). "Anti-gp210 and anti-centromere antibodies are different risk factors for the progression of primary biliary cirrhosis". Hepatology 45 (1): 118–27. doi:10.1002/hep.21472. PMID 17187436.