Stage-specific action of matrix metalloproteinases influences progressive hereditary kidney disease
2006-04 | journal article; research paper
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Stage-specific action of matrix metalloproteinases influences progressive hereditary kidney disease
Zeisberg, M. ; Khurana, M.; Rao, V. H.; Cosgrove, D.; Rougier, J.-P.; Werner, M. C. & Shield, C. F. et al. (2006)
PLoS Medicine, 3(4) art. e100. DOI: https://doi.org/10.1371/journal.pmed.0030100
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Details
- Authors
- Zeisberg, Michael ; Khurana, Mona; Rao, Velidi H.; Cosgrove, Dominic; Rougier, Jean-Philippe; Werner, Michelle C.; Shield, Charles F.; Werb, Zena; Kalluri, Raghu
- Abstract
- Glomerular basement membrane (GBM), a key component of the blood-filtration apparatus in the in the kidney, is formed through assembly of type IV collagen with laminins, nidogen, and sulfated proteoglycans. Mutations or deletions involving alpha3(IV), alpha4(IV), or alpha5(IV) chains of type IV collagen in the GBM have been identified as the cause for Alport syndrome in humans, a progressive hereditary kidney disease associated with deafness. The pathological mechanisms by which such mutations lead to eventual kidney failure are not completely understood.
- Issue Date
- April-2006
- Journal
- PLoS Medicine
- ISSN
- 1549-1676
- Language
- English