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Characterization of anti-GBM antibodies involved in Goodpasture's syndrome.
Kidney Int. 1994 Sep; 46(3):823-9.KI

Abstract

Goodpasture's syndrome is a life threatening autoimmune kidney disease. The patients have autoantibodies to the glomerular basement membrane, which are specific for the C-terminal domain of type IV collagen (NC1). The major antigen has been localized to the alpha 3 (IV)-chain. We have investigated sera from 44 patients with anti-NC1 antibodies. The quantity of antibodies to four different alpha(IV)-chains of type IV collagen was measured with direct ELISA. We used affinity chromatography to separate the antibodies and their specificities were studied with ELISA. The results show that about 1% of the patients total IgG are anti-NC1 antibodies and that 90% of these antibodies are specific for the alpha 3(IV)-chain. Antibodies to the other alpha(IV)-chains were found in 80% of the patients. Furthermore, affinity purified anti-alpha 3(IV) antibodies from one patient were inhibited by antibodies from the other patients, from 4 to 72%. The antibodies, from 39 of the patients, were inhibited by a monoclonal antibody against the alpha 3(IV)-chain. The results indicate that patients with Goodpasture's syndrome can have antibodies to most of the alpha(IV)-chains, while the majority of anti-NC1 antibodies are restricted to the alpha 3(IV)-chain. Moreover the number of epitopes seems to be limited and the majority of the antibodies from most patients are against one single epitope on the alpha 3(IV)-chain.

Authors+Show Affiliations

Department of Nephrology, University Hospital, Lund, Sweden.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

7527877

Citation

Hellmark, T, et al. "Characterization of anti-GBM Antibodies Involved in Goodpasture's Syndrome." Kidney International, vol. 46, no. 3, 1994, pp. 823-9.
Hellmark T, Johansson C, Wieslander J. Characterization of anti-GBM antibodies involved in Goodpasture's syndrome. Kidney Int. 1994;46(3):823-9.
Hellmark, T., Johansson, C., & Wieslander, J. (1994). Characterization of anti-GBM antibodies involved in Goodpasture's syndrome. Kidney International, 46(3), 823-9.
Hellmark T, Johansson C, Wieslander J. Characterization of anti-GBM Antibodies Involved in Goodpasture's Syndrome. Kidney Int. 1994;46(3):823-9. PubMed PMID: 7527877.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Characterization of anti-GBM antibodies involved in Goodpasture's syndrome. AU - Hellmark,T, AU - Johansson,C, AU - Wieslander,J, PY - 1994/9/1/pubmed PY - 1994/9/1/medline PY - 1994/9/1/entrez SP - 823 EP - 9 JF - Kidney international JO - Kidney Int VL - 46 IS - 3 N2 - Goodpasture's syndrome is a life threatening autoimmune kidney disease. The patients have autoantibodies to the glomerular basement membrane, which are specific for the C-terminal domain of type IV collagen (NC1). The major antigen has been localized to the alpha 3 (IV)-chain. We have investigated sera from 44 patients with anti-NC1 antibodies. The quantity of antibodies to four different alpha(IV)-chains of type IV collagen was measured with direct ELISA. We used affinity chromatography to separate the antibodies and their specificities were studied with ELISA. The results show that about 1% of the patients total IgG are anti-NC1 antibodies and that 90% of these antibodies are specific for the alpha 3(IV)-chain. Antibodies to the other alpha(IV)-chains were found in 80% of the patients. Furthermore, affinity purified anti-alpha 3(IV) antibodies from one patient were inhibited by antibodies from the other patients, from 4 to 72%. The antibodies, from 39 of the patients, were inhibited by a monoclonal antibody against the alpha 3(IV)-chain. The results indicate that patients with Goodpasture's syndrome can have antibodies to most of the alpha(IV)-chains, while the majority of anti-NC1 antibodies are restricted to the alpha 3(IV)-chain. Moreover the number of epitopes seems to be limited and the majority of the antibodies from most patients are against one single epitope on the alpha 3(IV)-chain. SN - 0085-2538 UR - https://www.unboundmedicine.com/medline/citation/7527877/Characterization_of_anti_GBM_antibodies_involved_in_Goodpasture's_syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0085-2538(15)58619-5 DB - PRIME DP - Unbound Medicine ER -