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 |
| AssociatedDisease(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
- ↑ 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.
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .

