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Research Article Free access | 10.1172/JCI113057

Alport familial nephritis. Absence of 28 kilodalton non-collagenous monomers of type IV collagen in glomerular basement membrane.

M M Kleppel, C E Kashtan, R J Butkowski, A J Fish, and A F Michael

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Published July 1, 1987 - More info

Published in Volume 80, Issue 1 on July 1, 1987
J Clin Invest. 1987;80(1):263–266. https://doi.org/10.1172/JCI113057.
© 1987 The American Society for Clinical Investigation
Published July 1, 1987 - Version history
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Abstract

Alport-type familial nephritis (FN), a genetic disorder, results in progressive renal insufficiency and sensorineural hearing loss. Immunochemical and biochemical analyses of the non-collagenous (NC1) domain of type IV collagen isolated from the glomerular basement membranes (GBM) of three males with this disease demonstrate absence of the normally occurring 28-kilodalton (kD) NC1 monomers, but persistence of the 26- and 24-kD monomeric subunits derived from alpha 1 and 2 (both type IV) collagen chains, respectively.

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