Decreased intracellular ATP content and intact mitochondrial energy generating capacity in human cystinotic fibroblasts (Q24302197)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:16439594%20AND%20SRC:MED&resulttype=core&format=json

16 January 2020

title

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

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:16439594%20AND%20SRC:MED&resulttype=core&format=json

Cystinosin, lysosomal cystine transporter

16 January 2020

main subject

1 reference

GOA release 2020-03-11

ATP metabolic process

1 reference

GOA release 2020-03-11

1 reference

3

1 reference

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16 January 2020

Peter H Willems

6

1 reference

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16 January 2020

Henk J Blom

8

1 reference

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16 January 2020

1

Elena N Levtchenko

1 reference

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16 January 2020

Lambertus van den Heuvel

9

object named as

Lambertus P van den Heuvel

1 reference

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16 January 2020

2

Martijn J G Wilmer

1 reference

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16 January 2020

author name string

Jan B Koenderink

4

1 reference

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16 January 2020

Henk-Jan Visch

5

1 reference

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16 January 2020

Adriana de Graaf-Hess

7

1 reference

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16 January 2020

Leo A Monnens

10

1 reference

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16 January 2020

language of work or name

English

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publication date

1 February 2006

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16 January 2020

1 reference

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16 January 2020

volume

59

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16 January 2020

issue

2

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16 January 2020

page(s)

287-292

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Cystinosin, the protein defective in cystinosis, is a H(+)-driven lysosomal cystine transporter

16 January 2020

cites work

1 reference

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21 January 2018

1 reference

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Effect of cystine dimethylester on renal solute handling and isolated renal tubule transport in the rat: a new model of the Fanconi syndrome.

21 January 2018

1 reference

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Intracellular cystine loading inhibits transport in the rabbit proximal convoluted tubule

21 January 2018

1 reference

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

21 January 2018

1 reference

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The Fanconi syndrome of cystinosis: insights into the pathophysiology

21 January 2018

1 reference

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Comparison of cystine determination in mixed leukocytes vs polymorphonuclear leukocytes for diagnosis of cystinosis and monitoring of cysteamine therapy

21 January 2018

1 reference

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96-well plate assay for sublethal metabolic activity

21 January 2018

1 reference

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Estimation of NADH oxidation in human skeletal muscle mitochondria.

21 January 2018

1 reference

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Inhibition of mitochondrial Na+-Ca2+ exchange restores agonist-induced ATP production and Ca2+ handling in human complex I deficiency.

21 January 2018

1 reference

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Mitochondrial priming modifies Ca2+ oscillations and insulin secretion in pancreatic islets

21 January 2018

1 reference

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Regulation of mitochondrial ATP synthesis by calcium: evidence for a long-term metabolic priming

21 January 2018

1 reference

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Isolated complex I deficiency in children: clinical, biochemical and genetic aspects

21 January 2018

1 reference

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Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency

21 January 2018

1 reference

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LLC-PK(1) cells maintained in a new perfusion cell culture system exhibit an improved oxidative metabolism

21 January 2018

1 reference

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Inhibition of Na(+)-dependent transporters in cystine-loaded human renal cells: electrophysiological studies on the Fanconi syndrome of cystinosis

21 January 2018

1 reference

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The leucocyte chemotactic function in patients with ulcerative colitis

21 January 2018

1 reference

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

21 January 2018

1 reference

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Glutathione precursors replenish decreased glutathione pool in cystinotic cell lines

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196334.46940.54

Altered status of glutathione and its metabolites in cystinotic cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196334.46940.54

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

21 January 2018

1 reference

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Rupture of rat liver lysosomes mediated by L-amino acid esters

21 January 2018

1 reference

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7 January 2021

Differential investigation of the capacity of succinate oxidation in human skeletal muscle

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196334.46940.54

7 January 2021

Use of fibroblast and lymphoblast cultures for detection of respiratory chain defects

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196334.46940.54

7 January 2021

Skin storage of cystine and premature skin ageing in cystinosis

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196334.46940.54

7 January 2021

Cystine: Compartmentalization within Lysosomes in Cystinotic Leukocytes

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196334.46940.54

7 January 2021

Lethal infantile mitochondrial disease with isolated complex I deficiency in fibroblasts but with combined complex I and IV deficiencies in muscle

1 reference

https://api.crossref.org/works/10.1203%2F01.PDR.0000196334.46940.54

7 January 2021

10.1203/01.PDR.0000196334.46940.54

Identifiers

DOI

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:16439594%20AND%20SRC:MED&resulttype=core&format=json

Dimensions Publication ID

16 January 2020

0 references

1 reference

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