Anti-glycoprotein-210 antibodies
| Autoantibody | |
|---|---|
| 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
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]
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.