Mitochondrial complex V expression and activity in cystinotic fibroblasts

Martijn J. Wilmer; Lambertus P. Van Den Heuvel; Richard J. Rodenburg; Rutger O. Vogel; Leo G. Nijtmans; Leo A. Monnens; Elena N. Levtchenko

doi:https://doi.org/10.1203/PDR.0b013e318183fd67

Mitochondrial complex V expression and activity in cystinotic fibroblasts

Martijn J. Wilmer, Lambertus P. Van Den Heuvel, Richard J. Rodenburg, Rutger O. Vogel, Leo G. Nijtmans, Leo A. Monnens, Elena N. Levtchenko

Research output: Contribution to journal › Article › Academic › peer-review

15 Citations (Scopus)

Abstract

Alterations in ATP metabolism have been proposed to be involved in the pathogenesis of cystinosis, the most common form of inherited Fanconi syndrome. A recent study showed normal activity of respiratory chain complexes I-IV with decreased ATP levels in cystinotic fibroblasts. Here, we show normal complex V expression and activity in mitochondria of cystinotic fibroblasts. This indicates that alterations in mitochondrial oxidative phosphorylation enzymes are not responsible for ATP decrease in cystinotic fibroblasts.

Original language	English
Pages (from-to)	495-497
Number of pages	3
Journal	Pediatric Research
Volume	64
Issue number	5
DOIs	https://doi.org/10.1203/PDR.0b013e318183fd67
Publication status	Published - Nov 2008
Externally published	Yes

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abstract = "Alterations in ATP metabolism have been proposed to be involved in the pathogenesis of cystinosis, the most common form of inherited Fanconi syndrome. A recent study showed normal activity of respiratory chain complexes I-IV with decreased ATP levels in cystinotic fibroblasts. Here, we show normal complex V expression and activity in mitochondria of cystinotic fibroblasts. This indicates that alterations in mitochondrial oxidative phosphorylation enzymes are not responsible for ATP decrease in cystinotic fibroblasts.",

author = "Wilmer, {Martijn J.} and {Van Den Heuvel}, {Lambertus P.} and Rodenburg, {Richard J.} and Vogel, {Rutger O.} and Nijtmans, {Leo G.} and Monnens, {Leo A.} and Levtchenko, {Elena N.}",

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T1 - Mitochondrial complex V expression and activity in cystinotic fibroblasts

AU - Wilmer, Martijn J.

AU - Van Den Heuvel, Lambertus P.

AU - Rodenburg, Richard J.

AU - Vogel, Rutger O.

AU - Nijtmans, Leo G.

AU - Monnens, Leo A.

AU - Levtchenko, Elena N.

PY - 2008/11

Y1 - 2008/11

N2 - Alterations in ATP metabolism have been proposed to be involved in the pathogenesis of cystinosis, the most common form of inherited Fanconi syndrome. A recent study showed normal activity of respiratory chain complexes I-IV with decreased ATP levels in cystinotic fibroblasts. Here, we show normal complex V expression and activity in mitochondria of cystinotic fibroblasts. This indicates that alterations in mitochondrial oxidative phosphorylation enzymes are not responsible for ATP decrease in cystinotic fibroblasts.

AB - Alterations in ATP metabolism have been proposed to be involved in the pathogenesis of cystinosis, the most common form of inherited Fanconi syndrome. A recent study showed normal activity of respiratory chain complexes I-IV with decreased ATP levels in cystinotic fibroblasts. Here, we show normal complex V expression and activity in mitochondria of cystinotic fibroblasts. This indicates that alterations in mitochondrial oxidative phosphorylation enzymes are not responsible for ATP decrease in cystinotic fibroblasts.

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U2 - https://doi.org/10.1203/PDR.0b013e318183fd67

DO - https://doi.org/10.1203/PDR.0b013e318183fd67

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SN - 0031-3998

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SP - 495

EP - 497

JO - Pediatric Research

JF - Pediatric Research

IS - 5

ER -

Mitochondrial complex V expression and activity in cystinotic fibroblasts

Abstract

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