(Q46913688)

26 December 2017

title

Impaired activity of the gamma-glutamyl cycle in nephropathic cystinosis fibroblasts (English)

1 reference

26 December 2017

Abderhalden–Kaufmann–Lignac syndrome

main subject

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2

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26 December 2017

Fiorella Piemonte

4

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26 December 2017

author name string

Liliana Mannucci

1

1 reference

26 December 2017

Cristiano Rizzo

3

1 reference

26 December 2017

Gianfranco Rizzoni

5

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26 December 2017

Francesco Emma

6

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26 December 2017

language of work or name

English

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publication date

1 February 2006

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26 December 2017

published in

Pediatric Research

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26 December 2017

volume

59

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26 December 2017

issue

2

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26 December 2017

page(s)

332-335

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26 December 2017

https://scigraph.springernature.com/pub.10.1203/01.pdr.0000196370.57200.da

exact match

0 references

cites work

Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

A novel gene encoding an integral membrane protein is mutated in nephropathic cystinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

Mutational spectrum of the CTNS gene in Italy

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

The use of skin fibroblast cultures in the detection of respiratory chain defects in patients with lacticacidemia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

The Fanconi syndrome of cystinosis: insights into the pathophysiology

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

Role of adenosine triphosphate (ATP) and NaK ATPase in the inhibition of proximal tubule transport with intracellular cystine loading.

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

Intracellular cystine loading causes proximal tubule respiratory dysfunction: effect of glycine

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

A cysteine-specific lysosomal transport system provides a major route for the delivery of thiol to human fibroblast lysosomes: possible role in supporting lysosomal proteolysis

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

Disulphide reduction in lysosomes. The role of cysteine

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

Glutathione precursors replenish decreased glutathione pool in cystinotic cell lines

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

Glycosaminoglycans reduce oxidative damage induced by copper (Cu+2), iron (Fe+2) and hydrogen peroxide (H2O2) in human fibroblast cultures

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

Mitochondrial damage in muscle occurs after marked depletion of glutathione and is prevented by giving glutathione monoester

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

Lysosomal cystine storage augments apoptosis in cultured human fibroblasts and renal tubular epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

Altered oxidative metabolism, motility, and adherence in phagocytic cells from cystinotic children

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

Neurologic complications in long-standing nephropathic cystinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

Beta-cell glucose toxicity, lipotoxicity, and chronic oxidative stress in type 2 diabetes

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196370.57200.DA

10.1203/01.PDR.0000196370.57200.DA

21 January 2018

Identifiers

DOI

1 reference

26 December 2017

Dimensions Publication ID

1008853590

0 references

1 reference

26 December 2017