Literature DB >> 8068003

The kinetic basis of threshold effects observed in mitochondrial diseases: a systemic approach.

T Letellier1, R Heinrich, M Malgat, J P Mazat.   

Abstract

Threshold effects in the expression of metabolic diseases have often been observed in mitochondrial pathologies, i.e. the clinical demonstration of the disease appears only when the activity of a step has been reduced to a rather low level. We show experimentally that an inhibition of cytochrome c oxidase activity by cyanide, simulating a defect in this step, leads to a decrease in mitochondrial respiration which then exhibits a threshold behaviour similar to that observed in mitochondrial diseases. We discuss this behaviour in terms of metabolic control theory and construct a mathematical model simulating this behaviour.

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Year:  1994        PMID： 8068003      PMCID： PMC1137205          DOI： 10.1042/bj3020171

Source DB:  PubMed          Journal:  Biochem J        ISSN： 0264-6021            Impact factor:   3.857


  17 in total

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Review 4.  Control of mitochondrial respiration.

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Journal:  Biochem J       Date:  1981-11-15       Impact factor: 3.857

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Authors:  H J Flint; D J Porteous; H Kacser
Journal:  Biochem J       Date:  1980-07-15       Impact factor: 3.857

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Journal:  Brain       Date:  1982-09       Impact factor: 13.501
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  37 in total

1.  Tissue variation in the control of oxidative phosphorylation: implication for mitochondrial diseases.

Authors:  R Rossignol; T Letellier; M Malgat; C Rocher; J P Mazat
Journal:  Biochem J       Date:  2000-04-01       Impact factor: 3.857

2.  Effect of 'binary mitochondrial heteroplasmy' on respiration and ATP synthesis: implications for mitochondrial diseases.

Authors:  B Korzeniewski; M Malgat; T Letellier; J P Mazat
Journal:  Biochem J       Date:  2001-08-01       Impact factor: 3.857

Review 3.  Mitochondrial threshold effects.

Authors:  Rodrigue Rossignol; Benjamin Faustin; Christophe Rocher; Monique Malgat; Jean-Pierre Mazat; Thierry Letellier
Journal:  Biochem J       Date:  2003-03-15       Impact factor: 3.857

Review 4.  Some observations upon biochemical causes of ataxia and a new disease entity ubiquinone, CoQ10 deficiency.

Authors:  John M Land; Simon J R Heales; Andrew J Duncan; Iain P Hargreaves
Journal:  Neurochem Res       Date:  2006-12-21       Impact factor: 3.996

5.  PGC-1alpha/beta upregulation is associated with improved oxidative phosphorylation in cells harboring nonsense mtDNA mutations.

Authors:  Sarika Srivastava; John N Barrett; Carlos T Moraes
Journal:  Hum Mol Genet       Date:  2007-03-06       Impact factor: 6.150

6.  Metabolic control analysis and mitochondrial pathologies.

Authors:  T Letellier; M Malgat; R Rossignol; J P Mazat
Journal:  Mol Cell Biochem       Date:  1998-07       Impact factor: 3.396

7.  In vivo control of respiration by cytochrome c oxidase in wild-type and mitochondrial DNA mutation-carrying human cells.

Authors:  G Villani; G Attardi
Journal:  Proc Natl Acad Sci U S A       Date:  1997-02-18       Impact factor: 11.205

Review 8.  Mitochondria, OxPhos, and neurodegeneration: cells are not just running out of gas.

Authors:  Estela Area-Gomez; Cristina Guardia-Laguarta; Eric A Schon; Serge Przedborski
Journal:  J Clin Invest       Date:  2019-01-02       Impact factor: 14.808

9.  Effect of enzyme deficiencies on oxidative phosphorylation: from isolated mitochondria to intact tissues. Theoretical studies.

Authors:  Bernard Korzeniewski
Journal:  Mol Biol Rep       Date:  2002       Impact factor: 2.316

10.  Decline in cytochrome c oxidase activity in rat-brain mitochondria with aging. Role of peroxidized cardiolipin and beneficial effect of melatonin.

Authors:  Giuseppe Petrosillo; Valentina De Benedictis; Francesca M Ruggiero; Giuseppe Paradies
Journal:  J Bioenerg Biomembr       Date:  2013-03-15       Impact factor: 2.945
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