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 Medicine3(4) art. e100​.​ DOI: https://doi.org/10.1371/journal.pmed.0030100 

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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

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