We are analyzing https://link.springer.com/article/10.1007/s00467-006-0005-x.

Title:
Glutathione depletion and increased apoptosis rate in human cystinotic proximal tubular cells | Pediatric Nephrology
Description:
We have determined levels of glutathione (GSH), ATP, mitochondrial complex activity and apoptosis rate in proximal tubular cells (PTCs) exfoliated from urine in cystinotic (n=9) and control (n=9) children. Intracellular GSH was significantly depleted in cystinotic PTCs compared with controls (6.8 nmol GSH/mg protein vs 11.8 nmol GSH/mg protein; P<0.001), but there were no significant differences in mitochondrial complex activities or ATP levels under basal conditions. Cystinotic PTCs showed significantly increased apoptosis rate. After PTCs had been stressed by hypoxia, there was further depletion of GSH in cystinotic and control PTCs (2.4 nmol GSH/mg protein vs 7.2 nmol GSH/mg protein; P<0.001). Hypoxic stress led to increased complex I and complex IV activities in control but not in cystinotic PTCs. ATP levels were significantly reduced in cystinotic PTCs after hypoxic stress (12.2 nmol/mg protein vs 26.9 nmol/mg protein; P<0.001). GSH depletion occurs in this in vitro model of cystinotic PTCs, is exaggerated by hypoxic stress and may contribute to reduced ATP and failure to increase complex I/IV activities. Apoptotic rate is also increased, and these mechanisms may contribute to cellular dysfunction in cultured, human cystinotic PTCs.
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Keywords {🔍}

article, google, scholar, cas, pubmed, glutathione, cystinotic, cystinosis, mitochondrial, protein, apoptosis, cells, nephrol, cystine, ptcs, cell, proximal, pediatr, renal, depletion, human, tubular, heales, vant, hoff, complex, res, fanconi, syndrome, increased, laube, atp, stress, access, mitochondria, london, cytochrome, privacy, cookies, content, rate, hargreaves, model, biol, cherqui, antignac, soc, kidney, university, hospital,

Topics {✒️}

month download article/chapter nitric oxide-mediated induction mitochondrial death/life regulator tiel/lacz metanephric kidneys high-performance liquid chromatography proximal tubular cells cytochrome b-c1 particle na-k-atpase polarity cellular glutathione concentration proximal tubule transport 8 nmol gsh/mg protein 4 nmol gsh/mg protein 2 nmol gsh/mg protein renal fanconi syndrome full article pdf privacy choices/manage cookies related subjects escherichia coli chemical-induced apoptosis beef heart mitochondria nrk-52e cells pediatric kidney tubules experimental fanconi syndrome human cystinotic ptcs lysosomal membrane transport cystinotic human fibroblasts cystinotic cell lines mitochondrial glutathione transporter 2 nmol/mg protein 9 nmol/mg protein kidney epithelial cells increased apoptosis rate acid-soluble cystine cell death impaired mitochondrial function hypoxic stress led intralysosomal cystine accumulation european economic area na+-dependent transporters darley-usmar vm γ-glutamyl cycle balkan endemic nephropathy quantitatively distinct microparticles spectrin-ankyrin complex integral membrane protein intracellular cystine loading gsh depletion occurs mitochondrial complex activity mitochondrial dna depletion cystinotic ptcs compared

Questions {❓}

Gegg ME, Beltran B, Salas-Pino S, Bolanos JP, Clark JB, Moncada S, Heales SJ (2003) Differential effect of nitric oxide on glutathione metabolism and mitochondrial function in astrocytes and neurones: implications for neuroprotection/neurodegeneration?

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         headline:Glutathione depletion and increased apoptosis rate in human cystinotic proximal tubular cells
         description:We have determined levels of glutathione (GSH), ATP, mitochondrial complex activity and apoptosis rate in proximal tubular cells (PTCs) exfoliated from urine in cystinotic (n=9) and control (n=9) children. Intracellular GSH was significantly depleted in cystinotic PTCs compared with controls (6.8 nmol GSH/mg protein vs 11.8 nmol GSH/mg protein; P<0.001), but there were no significant differences in mitochondrial complex activities or ATP levels under basal conditions. Cystinotic PTCs showed significantly increased apoptosis rate. After PTCs had been stressed by hypoxia, there was further depletion of GSH in cystinotic and control PTCs (2.4 nmol GSH/mg protein vs 7.2 nmol GSH/mg protein; P<0.001). Hypoxic stress led to increased complex I and complex IV activities in control but not in cystinotic PTCs. ATP levels were significantly reduced in cystinotic PTCs after hypoxic stress (12.2 nmol/mg protein vs 26.9 nmol/mg protein; P<0.001). GSH depletion occurs in this in vitro model of cystinotic PTCs, is exaggerated by hypoxic stress and may contribute to reduced ATP and failure to increase complex I/IV activities. Apoptotic rate is also increased, and these mechanisms may contribute to cellular dysfunction in cultured, human cystinotic PTCs.
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      headline:Glutathione depletion and increased apoptosis rate in human cystinotic proximal tubular cells
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