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| IMMUNOCHEMICAL STUDIES OF THE ALPORT ANTIGEN |
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| Author(s): KLEPPEL MM, FAN WW, CHEONG HI, KASHTAN CE, MICHAEL AF |
| Source: KIDNEY INTERNATIONAL Volume: 41 Issue: 6 Pages: 1629-1637 Published: JUN 1992 |
| Times Cited: 48 References: 32 |
| Abstract: The Alport antigen, a component of normal glomerular basement membranes (GBM) which is absent in Alport familial nephritis, is characterized as a 26 kD non-collagenous (NC1) peptide identified by a monoclonal antibody (Mab A7) and an Alport alloantibody. Both antibodies discriminate X-linkage of the Alport defect using indirect immunofluorescence of hemizygous and heterozygous Alport GBM and epidermal basement membrane (EBM). Immunoblotting of SDS-PAGE gels of collagenase-digested Alport renal BM shows absence of monomeric and dimeric components of the Alport antigen, alpha-3(IV) NC1, and alpha-4(IV) NC1. By immunoprecipitation experiments with specific antibodies, the Alport antigen is distinct from the 26 kD NC1 peptide derived from alpha-1(IV). The monoclonal antibody to the Alport antigen and rabbit antiserum to a non-consensus sequence of alpha-5(IV) NC1 react similarly by immunofluorescence with normal kidney and both fail to bind to Alport renal BM. Two dimension Western blots of collagenase-digested BM show that the anti-Alport antigen and the ant-alpha-5(IV) NC1 react similarly with monomeric and dimeric components of BM collagen. These studies are consistent with the likelihood that the Alport antigen and alpha-5(IV) NC1 are the same or are highly homologous molecules. The precise relationship will require characterization of alpha-5(IV) NC1 protein and determination of the nucleotide sequence of the Alport antigen. The associated absence of alpha-3(IV) NC1 and alpha-4(IV) (NC1) from Alport BM is consistent with other observations for a molecular association of these chains in a novel collagen network. |
| Document Type: Article |
| Language: English |
| Reprint Address: KLEPPEL, MM (reprint author), UNIV MINNESOTA, SCH MED, DEPT PEDIAT, BOX 491 UMHC, MINNEAPOLIS, MN 55455 USA |
Addresses:
1. UNIV MINNESOTA, SCH MED, DEPT LAB MED & PATHOL, MINNEAPOLIS, MN 55455 USA |
| Publisher: BLACKWELL SCIENCE INC, 350 MAIN ST, MALDEN, MA 02148 |
| Subject Category: Urology & Nephrology |
| IDS Number: HW904 |
| ISSN: 0085-2538 |
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