线粒体疾病中观察到的阈值效应的动力学基础：一种系统方法。
The kinetic basis of threshold effects observed in mitochondrial diseases: a systemic approach.
作者信息
Letellier T, Heinrich R, Malgat M, Mazat J P
机构信息
Université Bordeaux II, France.
出版信息
Biochem J. 1994 Aug 15;302 ( Pt 1)(Pt 1):171-4. doi: 10.1042/bj3020171.
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.
摘要
在线粒体病理学中经常观察到代谢疾病表达中的阈值效应，即只有当某一步骤的活性降低到相当低的水平时，疾病的临床症状才会显现出来。我们通过实验表明，用氰化物抑制细胞色素c氧化酶活性，模拟该步骤中的缺陷，会导致线粒体呼吸作用下降，进而表现出与线粒体疾病中观察到的类似的阈值行为。我们根据代谢控制理论讨论这种行为，并构建一个模拟这种行为的数学模型。
相似文献
1
The kinetic basis of threshold effects observed in mitochondrial diseases: a systemic approach.
Biochem J. 1994 Aug 15;302 ( Pt 1)(Pt 1):171-4. doi: 10.1042/bj3020171.
2
Photoreactivation of the cytochrome oxidase complex with cyanide: the reaction of heme a3 photoreduction.
Membr Cell Biol. 1998;12(5):743-54.
3
Differences in flux control and reserve capacity of cytochrome c oxidase (COX) in human skeletal muscle and brain suggest different metabolic effects of mild COX deficiencies.
Mol Biol Rep. 2002;29(1-2):89-92. doi: 10.1023/a:1020374924550.
4
Metabolic control analysis and mitochondrial pathologies.
Mol Cell Biochem. 1998 Jul;184(1-2):409-17.
5
[Membrane control of the photodeblocking reaction of cytochrome oxidase in the mitochondrial electron transport chain].
Biofizika. 1993 Nov-Dec;38(6):1043-6.
6
A re-examination of the reactions of cyanide with cytochrome c oxidase.
Biochem J. 1984 May 15;220(1):57-66. doi: 10.1042/bj2200057.
7
Carbon monoxide specifically inhibits cytochrome c oxidase of human mitochondrial respiratory chain.
Pharmacol Toxicol. 2003 Sep;93(3):142-6. doi: 10.1034/j.1600-0773.2003.930306.x.
8
Cyanide inhibition and pyruvate-induced recovery of cytochrome c oxidase.
J Bioenerg Biomembr. 2010 Oct;42(5):395-403. doi: 10.1007/s10863-010-9307-6. Epub 2010 Aug 20.
9
The impact of the thermal sensitivity of cytochrome c oxidase on the respiration rate of Arctic charr red muscle mitochondria. pierre_blier@uqar.qc.ca.
J Comp Physiol B. 2001 Apr;171(3):247-53. doi: 10.1007/s003600000169.
10
Functional dynamic compartmentalization of respiratory chain intermediate substrates: implications for the control of energy production and mitochondrial diseases.
Int J Biochem Cell Biol. 2008;40(8):1543-54. doi: 10.1016/j.biocel.2007.11.023. Epub 2007 Dec 14.
引用本文的文献
1
Assessing the mitochondrial safety profile of the molnupiravir active metabolite, β-d-N4-hydroxycytidine (NHC), in the physiologically relevant HepaRG model.
Toxicol Res (Camb). 2024 Feb 7;13(1):tfae012. doi: 10.1093/toxres/tfae012. eCollection 2024 Feb.
2
Drug Drop Test: How to Quickly Identify Potential Therapeutic Compounds for Mitochondrial Diseases Using Yeast .
Int J Mol Sci. 2023 Jun 27;24(13):10696. doi: 10.3390/ijms241310696.
3
Transcriptomic Analyses of Neurotoxic Astrocytes Derived from Adult Triple Transgenic Alzheimer's Disease Mice.
J Mol Neurosci. 2023 Aug;73(7-8):487-515. doi: 10.1007/s12031-023-02105-2. Epub 2023 Jun 15.
4
Lipids uniquely alter the secondary structure and toxicity of amyloid beta 1-42 aggregates.
FEBS J. 2023 Jun;290(12):3203-3220. doi: 10.1111/febs.16738. Epub 2023 Feb 9.
5
Cytochrome c Oxidase Activity as a Metabolic Regulator in Pancreatic Beta-Cells.
Cells. 2022 Mar 8;11(6):929. doi: 10.3390/cells11060929.
6
Neuroprotective Effects of a Small Mitochondrially-Targeted Tetrapeptide Elamipretide in Neurodegeneration.
Front Integr Neurosci. 2022 Jan 17;15:747901. doi: 10.3389/fnint.2021.747901. eCollection 2021.
7
Multiple Mechanisms Regulate Eukaryotic Cytochrome C Oxidase.
Cells. 2021 Feb 28;10(3):514. doi: 10.3390/cells10030514.
8
Regulation of cytochrome c oxidase contributes to health and optimal life.
World J Biol Chem. 2020 Sep 27;11(2):52-61. doi: 10.4331/wjbc.v11.i2.52.
9
Mitochondria dysfunction in the pathogenesis of Alzheimer's disease: recent advances.
Mol Neurodegener. 2020 May 29;15(1):30. doi: 10.1186/s13024-020-00376-6.
10
Analysis of the Potential for -Hydroxycytidine To Inhibit Mitochondrial Replication and Function.
Antimicrob Agents Chemother. 2020 Jan 27;64(2). doi: 10.1128/AAC.01719-19.
本文引用的文献
1
The respiratory chain and oxidative phosphorylation.
Adv Enzymol Relat Subj Biochem. 1956;17:65-134. doi: 10.1002/9780470122624.ch2.
2
Succinate-cytochrome c reductase: assessment of its value in the investigation of defects of the respiratory chain.
Biochim Biophys Acta. 1993 Jun 19;1181(3):261-5. doi: 10.1016/0925-4439(93)90030-5.
3
Control of oxidative phosphorylation in rat muscle mitochondria: implications for mitochondrial myopathies.
Biochim Biophys Acta. 1993 Feb 8;1141(1):58-64. doi: 10.1016/0005-2728(93)90189-m.
4
5
The molecular basis of dominance.
Genetics. 1981 Mar-Apr;97(3-4):639-66. doi: 10.1093/genetics/97.3-4.639.
6
Mitochondrial encephalomyopathies: biochemical studies in two cases revealing defects in the respiratory chain.
Brain. 1982 Sep;105 (Pt 3):553-82. doi: 10.1093/brain/105.3.553.
7
Quantification of the contribution of various steps to the control of mitochondrial respiration.
J Biol Chem. 1982 Mar 25;257(6):2754-7.
8
Control of the flux in the arginine pathway of Neurospora crassa. Modulations of enzyme activity and concentration.
Biochem J. 1981 Nov 15;200(2):231-46. doi: 10.1042/bj2000231.
9
Control of the flux in the arginine pathway of Neurospora crassa. The flux from citrulline to arginine.
Biochem J. 1980 Jul 15;190(1):1-15. doi: 10.1042/bj1900001.
10
Control of mitochondrial respiration.
FEBS Lett. 1983 Jan 10;151(1):1-9. doi: 10.1016/0014-5793(83)80330-5.
Zotero 插件