Article
The swan-neck lesion: proximal tubular adaptation to oxidative stress in nephropathic cystinosis.
American journal of physiology. Renal physiology - 15 May 2015
Galarreta Carolina I, Forbes Michael S, Thornhill Barbara A, Antignac Corinne, Gubler Marie-Claire, Nevo Nathalie, Murphy Michael P, Chevalier Robert L
Abstract excerpt
Cystinosis is an inherited disorder resulting from a mutation in the CTNS gene, causing progressive proximal tubular cell flattening, the so-called swan-neck lesion (SNL), and eventual renal failure. To determine the role of oxidative stress in cystinosis, histologic sections of kidneys from C57BL/6 Ctns(-/-) and wild-type mice were examined by immunohistochemistry and morphometry from 1 wk to 20 mo of age....
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