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The redox status of cystinotic fibroblasts.

Abstract：

:A key unresolved question in the pathogenesis of phenotype development in nephropathic cystinosis is whether intralysosomal cystine, the hallmark of this lethal inborn error of metabolism, alters cytoplasmic redox potential. Variable findings on this issue have been reported. This study of fetal and non-fetal skin and lung-derived cystinotic fibroblasts compared to origin and age-matched normal control fibroblasts reveals that cystinotic cells do not exhibit redox perturbations. We find that the steady-state redox status as assessed by the [GSH]/[GSSG] ratio, an indicator of the intracellular redox poise, is unchanged in cystinotic cells. Furthermore, the dependence of the intracellular GSH and cysteine pool sizes and the [GSH]/[GSSG] ratio are similarly dependent on the two major sources of cysteine, i.e. the transsulfuration pathway and the plasma membrane cystine transporter, xc(-), in both cystinotic and control cells, and the presence of lysosomal cystine has no measurable effect on the redox status of these cells. Hence, mechanisms other than cytosolic redox perturbations are involved in the etiology of nephropathic cystinosis.

journal_name

Mol Genet Metab

journal_title

Molecular genetics and metabolism

authors

Vitvitsky V,Witcher M,Banerjee R,Thoene J

doi

10.1016/j.ymgme.2009.12.010

subject

Has Abstract

pub_date

2010-04-01 00:00:00

pages

384-8

issue

4

eissn

1096-7192

issn

1096-7206

pii

S1096-7192(09)00531-9

journal_volume

99

pub_type

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