Anti-glycoprotein-210 antibodies

Autoantibody(s)
Anti-glycoprotein-210
Autoantigen Isoform	Nucleoporin 210kDa
Autoantigen gene	NUP210
Affected organ(s)	Bile Duct
Associated 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]

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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

^ 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.
^ 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.
^ 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.
^ 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.
^ 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.
^ 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.

v • d • e Antibodies: autoantibodies
Anti-actin - Anti-apolipoprotein - Anti-cardiolipin - Anti-centromere - Anti-ganglioside - Anti-gp210 - Anti-mitochondrial antibodies - Anti-neutrophil cytoplasmic antibody - Anti-nuclear antibody - Anti-p62 - Anti-phopsholipid - Anti-sp100 - Anti-thrombin - Anti-topoisomerase - Anti-transglutaminase - Lupus anticoagulant - Rheumatoid factor -

Categories: Autoantibodies | Autoimmune diseases | Antibodies | PBC Autoantibodies

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