(Q46505867)

22 December 2017

title

Mitochondrial complex V expression and activity in cystinotic fibroblasts (English)

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22 December 2017

author

Richard J Rodenburg

3

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22 December 2017

author name string

Martijn J Wilmer

1

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22 December 2017

Lambertus P van den Heuvel

2

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22 December 2017

Rutger O Vogel

4

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22 December 2017

Leo G Nijtmans

5

1 reference

22 December 2017

Leo A Monnens

6

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22 December 2017

Elena N Levtchenko

7

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22 December 2017

language of work or name

English

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publication date

1 November 2008

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22 December 2017

published in

Pediatric Research

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22 December 2017

volume

64

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22 December 2017

issue

5

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22 December 2017

page(s)

495-497

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22 December 2017

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

cites work

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

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%2FPDR.0B013E318183FD67

The Fanconi syndrome of cystinosis: insights into the pathophysiology

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

Decreased intracellular ATP content and intact mitochondrial energy generating capacity in human cystinotic fibroblasts

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

Blue Native electrophoresis to study mitochondrial and other protein complexes

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

Glutathione precursors replenish decreased glutathione pool in cystinotic cell lines

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

Altered status of glutathione and its metabolites in cystinotic cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

Glutathione depletion and increased apoptosis rate in human cystinotic proximal tubular cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

Increased apoptosis in cystinotic fibroblasts and renal proximal tubule epithelial cells results from cysteinylation of protein kinase Cdelta.

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

Exfoliated human proximal tubular cells: a model of cystinosis and Fanconi syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

Elevated oxidized glutathione in cystinotic proximal tubular epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

Intralysosomal cystine accumulation in mice lacking cystinosin, the protein defective in cystinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

Urinary L-lactate excretion is increased in renal Fanconi syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

In vivo functional investigations of lactic acid in patients with respiratory chain disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2FPDR.0B013E318183FD67

10.1203/PDR.0B013E318183FD67

21 January 2018

Identifiers

DOI

1 reference

22 December 2017

Dimensions Publication ID

1038143817

0 references

1 reference

22 December 2017