Anti-glycoprotein-210 antibodies

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]

Autoantibody
Anti-glycoprotein-210
Autoantigen
Isoform		Nucleoporin 210kDa
Autoantigen geneNUP210
Affected organ(s)Bile Duct
Associated
Disease(s)		Primary biliary cirrhosis
HLA associationsDR2 (weak)

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.

Notes

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.

References
  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.
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