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Difference between revisions of "Rossignol 2003 Biochem J"

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{{Publication
{{Publication
|title=Rossignol R, Faustin B, Rocher C, Malgat M, Mazat JP, Letellier T (2003) Mitochondrial threshold effects. Biochem J 370: 751-762. Β 
|title=Rossignol R, Faustin B, Rocher C, Malgat M, Mazat JP, Letellier T (2003) Mitochondrial threshold effects. Biochem J 370:751-62.
|info=[https://www.ncbi.nlm.nih.gov/pubmed/12467494 PMID:12467494]
|authors=Rossignol R, Faustin B, Rocher C, Malgat M, Mazat JP, Letellier T
|authors=Rossignol R, Faustin B, Rocher C, Malgat M, Mazat JP, Letellier T
|year=2003
|year=2003
|journal=Biochem J
|journal=Biochem J
|abstract=The study of mitochondrial diseases has revealed dramatic variability in the phenotypic presentation of mitochondrial genetic defects. To attempt to understand this variability, different authors have studied energy metabolism in transmitochondrial cell lines carrying different proportions of various pathogenic mutations in their mitochondrial DNA. The same kinds of experiments have been performed on isolated mitochondria and on tissue biopsies taken from patients with mitochondrial diseases. The results have shown that, in most cases, phenotypic manifestation of the genetic defect occurs only when a threshold level is exceeded, and this phenomenon has been named the 'phenotypic threshold effect'. Subsequently, several authors showed that it was possible to inhibit considerably the activity of a respiratory chain complex, up to a critical value, without affecting the rate of mitochondrial respiration or ATP synthesis. This phenomenon was called the 'biochemical threshold effect'. More recently, quantitative analysis of the effects of various mutations in mitochondrial DNA on the rate of mitochondrial protein synthesis has revealed the existence of a 'translational threshold effect'. In this review these different mitochondrial threshold effects are discussed, along with their molecular bases and the roles that they play in the presentation of mitochondrial diseases.
|keywords=complementation, Metabolic Control Analysis, mitochondrial diseases, mitochondrial DNA, oxidative phosphorylation, threshold effect
|editor=[[Sumbalova Z]],
|mipnetlab=FR Bordeaux Letellier T, FR Bordeaux Rossignol R
}}
}}
== Cited by ==
{{Template:Cited by Gnaiger 2020 BEC MitoPathways}}
{{Labeling
{{Labeling
|preparations=Isolated Mitochondria
|preparations=Isolated mitochondria
|couplingstates=OXPHOS
|couplingstates=OXPHOS
|substratestates=CI, CII, CIV
|pathways=N, S, CIV
|additional=BEC 2020.2
}}
}}
* Referred to in [[Gnaiger_2012_MitoPathways]], Chapter 1.

Revision as of 16:58, 16 January 2021

Publications in the MiPMap
Rossignol R, Faustin B, Rocher C, Malgat M, Mazat JP, Letellier T (2003) Mitochondrial threshold effects. Biochem J 370:751-62.

Β» PMID:12467494

Rossignol R, Faustin B, Rocher C, Malgat M, Mazat JP, Letellier T (2003) Biochem J

Abstract: The study of mitochondrial diseases has revealed dramatic variability in the phenotypic presentation of mitochondrial genetic defects. To attempt to understand this variability, different authors have studied energy metabolism in transmitochondrial cell lines carrying different proportions of various pathogenic mutations in their mitochondrial DNA. The same kinds of experiments have been performed on isolated mitochondria and on tissue biopsies taken from patients with mitochondrial diseases. The results have shown that, in most cases, phenotypic manifestation of the genetic defect occurs only when a threshold level is exceeded, and this phenomenon has been named the 'phenotypic threshold effect'. Subsequently, several authors showed that it was possible to inhibit considerably the activity of a respiratory chain complex, up to a critical value, without affecting the rate of mitochondrial respiration or ATP synthesis. This phenomenon was called the 'biochemical threshold effect'. More recently, quantitative analysis of the effects of various mutations in mitochondrial DNA on the rate of mitochondrial protein synthesis has revealed the existence of a 'translational threshold effect'. In this review these different mitochondrial threshold effects are discussed, along with their molecular bases and the roles that they play in the presentation of mitochondrial diseases. β€’ Keywords: complementation, Metabolic Control Analysis, mitochondrial diseases, mitochondrial DNA, oxidative phosphorylation, threshold effect β€’ Bioblast editor: Sumbalova Z β€’ O2k-Network Lab: FR Bordeaux Letellier T, FR Bordeaux Rossignol R

Cited by

Gnaiger 2020 BEC MitoPathways
Gnaiger E (2020) Mitochondrial pathways and respiratory control. An introduction to OXPHOS analysis. 5th ed. Bioenerg Commun 2020.2. https://doi.org/10.26124/bec:2020-0002



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Preparation: Isolated mitochondria 


Coupling state: OXPHOS  Pathway: N, S, CIV 


BEC 2020.2