Nutritional Regulation of Mitochondrial Function

Vasam, Goutham; Reid, Kimberly J.; Burelle, Yan; Menzies, Keir J.

doi:10.1016/b978-0-12-811752-1.00004-3

Cited by 6 publications

(9 citation statements)

References 192 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The link reaction was mediated by a group of enzymes called the PDH complex. Acetyl CoA produced in the link reaction enters the Krebs cycle generating 3 NADH and 1 FADH ( 3 ). All the NADH and FADH produced were transported to the electron transport chain located at the mitochondrial matrix to produce ATP ( 4 ).…”

Section: Carbohydrate Metabolism Pathwaymentioning

confidence: 99%

“…The ketogenic diet is a diet that comprises mostly fat, moderate-to-low protein and low carbohydrate ( 3 ). Clinical Global Impression of Improvement was used for assessment and this diet has shown to improve health, and is an effective option, for PDH deficiency patients ( 12 ).…”

Section: Current Treatmments Of Pyruvate Dehydrogenase Deficiencymentioning

confidence: 99%

“…Individuals with PDH deficiency are often prescribed a ketogenic diet, which contains high fat but low carbohydrate ( 3 , 4 ). With this diet regime, patients obtain most of their metabolic fuels via fatty acid oxidation, rather than carbohydrate catabolism.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Comparison Between Dichloroacetate and Phenylbutyrate Treatment for Pyruvate Dehydrogenase Deficiency

Karissa¹,

Simpson

Dawson

et al. 2022

Br J Biomed Sci

View full text Add to dashboard Cite

Pyruvate dehydrogenase (PDH) deficiency is caused by a number of pathogenic variants and the most common are found in the PDHA1 gene. The PDHA1 gene encodes one of the subunits of the PDH enzyme found in a carbohydrate metabolism pathway involved in energy production. Pathogenic variants of PDHA1 gene usually impact the α-subunit of PDH causing energy reduction. It potentially leads to increased mortality in sufferers. Potential treatments for this disease include dichloroacetate and phenylbutyrate, previously used for other diseases such as cancer and maple syrup urine disease. However, not much is known about their efficacy in treating PDH deficiency. Effective treatment for PDH deficiency is crucial as carbohydrate is needed in a healthy diet and rice is the staple food for a large portion of the Asian population. This review analysed the efficacy of dichloroacetate and phenylbutyrate as potential treatments for PDH deficiency caused by PDHA1 pathogenic variants. Based on the findings of this review, dichloroacetate will have an effect on most PDHA1 pathogenic variant and can act as a temporary treatment to reduce the lactic acidosis, a common symptom of PDH deficiency. Phenylbutyrate can only be used on patients with certain pathogenic variants (p.P221L, p.R234G, p.G249R, p.R349C, p.R349H) on the PDH protein. It is hoped that the review would provide an insight into these treatments and improve the quality of lives for patients with PDH deficiency.

show abstract

Section: Carbohydrate Metabolism Pathwaymentioning

confidence: 99%

Section: Current Treatmments Of Pyruvate Dehydrogenase Deficiencymentioning

confidence: 99%

See 1 more Smart Citation

Comparison Between Dichloroacetate and Phenylbutyrate Treatment for Pyruvate Dehydrogenase Deficiency

Karissa¹,

Simpson

Dawson

et al. 2022

Br J Biomed Sci

View full text Add to dashboard Cite

show abstract

“…Similarly, micronutrients such as vitamins and minerals are essential for mitochondrial function and biological activities, often affecting mitochondrial health as antioxidants, cofactors or coenzymes, or components of mitochondrial biogenesis and/or metabolic functions. A detailed description of how macro-or micronutrients modulate mitochondrial health and the molecular mechanisms involved is beyond the scope of this review because it has been recently and exhaustively analysed (15,16) . The modulation of mitochondrial function and redox homeostasis by nutritional components is summarised in Table 1.…”

Section: Nutritional Regulation Of Mitochondrial Functionmentioning

confidence: 99%

Crosstalk between mitochondrial metabolism and oxidoreductive homeostasis: a new perspective for understanding the effects of bioactive dietary compounds

et al. 2019

View full text Add to dashboard Cite

Mitochondria play an important role in a number of fundamental cellular processes, including energy production, biosynthetic pathways and cellular oxidoreductive homeostasis (redox status), and their dysfunction can lead to numerous pathophysiological consequences. As the biochemical mechanisms orchestrating mitochondrial metabolism and redox homeostasis are functionally linked, mitochondria have been identified as a potential therapeutic target. Consequently, considerable effort has been made to evaluate the efficacy of natural compounds that modulate mitochondrial function. Molecules produced by plants (for example, polyphenols and isothiocyanates) have been shown to modulate mitochondrial metabolism/biogenesis and redox status; however, despite the existence of a functional link, few studies have considered the combined efficacy of these mitochondrial functions. The present review provides a complete overview of the molecular pathways involved in modulating mitochondrial metabolism/biogenesis and redox status. Crosstalk between these critical mechanisms is also discussed, whilst major data from the literature regarding their antioxidant abilities are described and critically analysed. We also provide a summary of recent evidence regarding the ability of several plant-derived compounds to target these mitochondrial functions. An in-depth understanding of the functional link between mitochondrial metabolism/biogenesis and redox status could facilitate the analysis of the biological effects of natural compounds as well as the development of new therapeutic approaches.

show abstract

“…We measured six enzymes of D. armandi mitochondrion, including NAD-malic enzyme (NAD-ME), NADPmalic enzyme (NADP-ME), mitochondrial isocitrate dehydrogenase (ICDHm), glutamate dehydrogenase (GDH), aconitase (ACO), and lipase (LPS). These enzymes play a pivotal regulation role in mitochondrial energy metabolism, such as the TCA cycle and fatty acid metabolism (Vasam et al, 2019). For instance, malic enzyme (ME) is a key enzyme regulating malic acid metabolismit plays a crucial role in catalyzing the reversible reaction for the oxidative decarboxylation of melamine, producing pyruvic acid and CO 2 , and reducing the reaction to NAD (P) + (Alvarez et al, 2012;Giordana et al, 2018;Rozova et al, 2019).…”

Section: Introductionmentioning

confidence: 99%

Effects of cold stress on metabolic regulation in the overwintering larvae of the Chinese white pine beetle, Dendroctonus armandi

Dai

Ning

et al. 2020

Entomologia Exp Applicata

View full text Add to dashboard Cite

The Chinese white pine beetle, Dendroctonus armandi Tsai & Li (Coleoptera: Curculionidae, Scolytinae), is considered the most destructive forest pest in the Qinling and Bashan Mountains of China. In recent years, winter temperature has dropped in these regions, and extremely low temperatures are hard to survive for insects. Cold hardiness becomes a crucial strategy because temperature change often leads to fluctuations in insect abundance, and the metabolism rate is a key index of resistance to cold in overwintering insects. Therefore, we investigated the relationship between the change in respiratory rate and the activity of metabolism-related mitochondrial enzymes in D. armandi larvae under cold conditions. We found that the respiratory rate decreased, and it was matched with the activity of glutamate dehydrogenase, aconitase, and lipase during overwintering. Among the various test times under cold conditions, the respiratory rate also decreased with decreasing temperature and increased under very low temperatures. At all cold stress periods, glutamate dehydrogenase and lipase showed increased activity at higher temperatures and decreased activity under lower temperatures, but the activity of NAD-malic enzyme, NADP-malic enzyme, mitochondrial isocitrate dehydrogenase, and aconitase were contrary. Under all low temperatures, the activity of enzymesexcept for NADP-malic enzyme, glutamate dehydrogenase, and lipaseincreased in short-term cold stress and decreased in long-term cold stress at 4, 0, −4, −6, −8, and −10°C. However, at −2°C, the activity of enzymes showed a decreasing trend in short-term treatments and an increasing trend in long-term treatments, except for mitochondrial isocitrate dehydrogenase. The results not only improve our understanding of the metabolic mechanism of cold adaptation in D. armandi, but also provide an important experimental basis for further study and biological pest control.

show abstract

Nutritional Regulation of Mitochondrial Function

Cited by 6 publications

References 192 publications

Comparison Between Dichloroacetate and Phenylbutyrate Treatment for Pyruvate Dehydrogenase Deficiency

Comparison Between Dichloroacetate and Phenylbutyrate Treatment for Pyruvate Dehydrogenase Deficiency

Crosstalk between mitochondrial metabolism and oxidoreductive homeostasis: a new perspective for understanding the effects of bioactive dietary compounds

Effects of cold stress on metabolic regulation in the overwintering larvae of the Chinese white pine beetle, Dendroctonus armandi

Contact Info

Product

Resources

About