Rossignol 2003 Biochem J: Difference between revisions
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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 370:751-62. | |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 | |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 | ||
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|keywords=complementation, Metabolic Control Analysis, mitochondrial diseases, mitochondrial DNA, oxidative phosphorylation, threshold effect | |keywords=complementation, Metabolic Control Analysis, mitochondrial diseases, mitochondrial DNA, oxidative phosphorylation, threshold effect | ||
|editor=[[Sumbalova Z]], | |editor=[[Sumbalova Z]], | ||
|mipnetlab=FR Bordeaux Letellier T, FR Bordeaux Rossignol R | |||
}} | }} | ||
== Cited by == | |||
{{Template:Cited by Gnaiger 2020 BEC MitoPathways}} | |||
{{Template:Cited by Krako Jakovljevic 2021 BEC PD}} | |||
{{Labeling | {{Labeling | ||
|preparations=Isolated mitochondria | |preparations=Isolated mitochondria | ||
|couplingstates=OXPHOS | |couplingstates=OXPHOS | ||
|pathways=N, S, CIV | |pathways=N, S, CIV | ||
|additional=BEC 2020.2 | |||
}} | }} | ||
Latest revision as of 12:25, 3 May 2022
Rossignol R, Faustin B, Rocher C, Malgat M, Mazat JP, Letellier T (2003) Mitochondrial threshold effects. Biochem J 370:751-62. |
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 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
- Krako Jakovljevic N, Ebanks B, Katyal G, Chakrabarti L, Markovic I, Moisoi N (2021) Mitochondrial homeostasis in cellular models of Parkinsonβs Disease. Bioenerg Commun 2021.2. https://doi.org/10.26124/bec:2021-0002
Labels:
Preparation: Isolated mitochondria
Coupling state: OXPHOS
Pathway: N, S, CIV
BEC 2020.2