|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 and are found within primary biliary cirrhosis (PBC) patients in high frequency. AGPA recognize the cytoplasmic oriented carboxyl terminus (tail) of the protein. 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. 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. PBC patients with potentially destructive AGPA have increased expression of Nup210 in the bile duct, a potential immune tolerance-escaping factor.
Anti-mitochondrial, anti-centromere 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.
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.
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