Anti-glycoprotein-210 antibodies

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Autoantibody(s)
Anti-glycoprotein-210
Autoantigen
Isoform
Nucleoporin 210kDa
Autoantigen gene NUP210
Affected organ(s) Bile Duct
Associated</br>Disease(s) 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 predicts 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.

Notes

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. 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. 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. 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. PMID 15710222. doi:10.1016/j.jhep.2004.11.016. 
  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. PMID 16337775. doi:10.1016/j.jaut.2005.10.007. 
  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. PMID 17187436. doi:10.1002/hep.21472. 

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