Anti-glycoprotein-210 antibodies
Encyclopedia
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.
Nucleoporin 210kDa
Nuclear pore glycoprotein-210 is an essential trafficking regulator in the eukaryotic nuclear pore complex. Gp-210 anchors the pore complex to the nuclear membrane...
and are found within primary biliary cirrhosis
Primary biliary cirrhosis
Primary biliary cirrhosis, often abbreviated PBC, is an autoimmune disease of the liver marked by the slow progressive destruction of the small bile ducts within the liver. When these ducts are damaged, bile builds up in the liver and over time damages the tissue. This can lead to scarring,...
(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
Immune tolerance
Immune tolerance or immunological tolerance is the process by which the immune system does not attack an antigen. It can be either 'natural' or 'self tolerance', in which the body does not mount an immune response to self antigens, or 'induced tolerance', in which tolerance to external antigens can...
-escaping factor.
Anti-mitochondrial
Anti-mitochondrial antibodies
Anti-mitochondrial antibodies are autoantibodies, consisting of immunoglobulins formed against mitochondria, primarily mitochondria in cells of the liver...
, anti-centromere
Anti-centromere antibodies
Anti-centromere antibodies occur in auto immune disorders; frequently in limited systemic scleroderma, , and, occasionally are found in the diffuse form of scleroderma...
and anti-p62 antibodies
Anti-p62 antibodies
Anti-p62 antibodies are found in a primary biliary cirrhosis. p62 is also more frequent in Stage IV primary biliary cirrhosis and is prognostic for severe disease. The autoantigen is the nucleoporin 62kDA protein....
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.