Antioxidants successfully reduce ROS production in propionic acidemia fibroblasts

Gallego, Lorena; Pérez, Belén; Ugarte, Magdalena; Desviat, Lourdes R.; Richard, Eva

doi:10.1016/j.bbrc.2014.08.091

Cited by 39 publications

(24 citation statements)

References 28 publications

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“…Moreover, understanding of the gene and enzyme structure has brought to our attention a number of possible gene therapies which are currently being studied in mice [117–119]. The field continues to explore the impact of antioxidants [79] and anaplerotic supplementation [120]. …”

Section: Resultsmentioning

confidence: 99%

“…Inflammation and reactive oxygen species (ROS) appear to be elevated in individuals with PA and their tissues [71, 79–81]. ROS levels assayed in fibroblasts from individuals with PA after antioxidant use, showed some improvement.…”

Section: Reviewmentioning

confidence: 99%

“…Trolox (a vitamin E analog which prevents lipid derived oxidant progression) also decreased ROS by 15–30%. In addition, resveratrol (inhibits lipid perioxidation) also showed a 30–40% decrease in ROS [79]. However, the traditional ROS scavengers including N-acetylcysteine (which re-generates glutathione) and melatonin (a direct free radical scavenger) were not successful in decreasing ROS implying that the best benefits are from stabilizers of mitochondrial function or preventers of lipid peroxidation and progression [79].…”

Section: Reviewmentioning

confidence: 99%

“…In addition, resveratrol (inhibits lipid perioxidation) also showed a 30–40% decrease in ROS [79]. However, the traditional ROS scavengers including N-acetylcysteine (which re-generates glutathione) and melatonin (a direct free radical scavenger) were not successful in decreasing ROS implying that the best benefits are from stabilizers of mitochondrial function or preventers of lipid peroxidation and progression [79]. Moreover, free radical production in PA-derived fibroblast appears to be associated JNK and p38 signaling pathways [80].…”

Section: Reviewmentioning

confidence: 99%

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Propionyl-CoA carboxylase – A review

Wongkittichote

Mew

Chapman

2017

Molecular Genetics and Metabolism

165

159

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Propionyl-CoA carboxylase (PCC) is the enzyme which catalyzes the carboxylation of propionyl-CoA to methylmalonyl-CoA and is encoded by the genes PCCA and PCCB to form a hetero-dodecamer. Dysfunction of PCC leads to the inherited metabolic disorder propionic acidemia, which can result in an affected individual presenting with metabolic acidosis, hyperammonemia, lethargy, vomiting and sometimes coma and death if not treated. Individuals with propionic acidemia also have a number of long term complications resulting from the dysfunction of the PCC enzyme. Here we present an overview of the current knowledge about the structure and function of PCC. We review an updated list of human variants which are published and provide an overview of the disease.

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Section: Resultsmentioning

confidence: 99%

Section: Reviewmentioning

confidence: 99%

Section: Reviewmentioning

confidence: 99%

Section: Reviewmentioning

confidence: 99%

See 2 more Smart Citations

Propionyl-CoA carboxylase – A review

Wongkittichote

Mew

Chapman

2017

Molecular Genetics and Metabolism

165

159

View full text Add to dashboard Cite

show abstract

“…In humans, resveratrol also improved glucose sensitivity in type 2 diabetic and healthy obese men (Timmers et al 2011; Bhatt et al 2012; Konings et al 2014), although other studies report no clinical effect of supplementation (Yoshino et al 2012; Poulsen et al 2013). No clinical studies have been performed yet with resveratrol in patients with inborn errors of metabolism, but in vitro studies reported improved metabolic function in fibroblasts of patients with mitochondrial fatty acid oxidation defects (Bastin et al 2011; Aires et al 2014), respiratory chain deficiencies (Lopes Costa et al 2014) and propionic acidemia (Gallego-Villar et al 2014). Although additional work is needed to elucidate the mechanisms underlying this restoration, it is likely due to an increase in residual activity of the defective enzyme, caused by PGC-1α-dependent mitochondrial biogenesis.…”

Section: Pharmacological Sirtuin Activation To Treat Inborn Errors Ofmentioning

confidence: 99%

Sirtuin activation as a therapeutic approach against inborn errors of metabolism

Bleeker

Houtkooper

2016

J of Inher Metab Disea

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Protein acylation has emerged as a large family of post translational modifications in which an acyl group can alter the function of a wide variety of proteins, especially in response to metabolic stress. The acylation state is regulated through reversible acylation/deacylation. Acylation occurs enzymatically or non-enzymatically, and responds to acyl-CoA levels. Deacylation on the other hand is controlled through the NAD+-dependent sirtuin proteins. In several inborn errors of metabolism (IEMs), accumulation of acyl-CoAs, due to defects in amino acid and fatty acid metabolic pathways, can lead to hyperacylation of proteins. This can have a direct effect on protein function and might play a role in pathophysiology. In this review we describe several mouse and cell models for IEM that display high levels of lysine acylation. Furthermore, we discuss how sirtuins serve as a promising therapeutic target to restore acylation state and could treat IEMs. In this context we examine several pharmacological sirtuin activators, such as resveratrol, NAD+ precursors and PARP and CD38 inhibitors.

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Nanoscale Optoregulation of Neural Stem Cell Differentiation by Intracellular Alteration of Redox Balance

Hassanpour-Tamrin

Taheri

Hasani-Sadrabadi

et al. 2017

Adv Funct Materials

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Regulation of stem cell (SC) fate, a decision between self-renewal and differentiation, is of immense importance in regenerative medicine and has been proven to be a powerful stimulus regulating many cell functions influencing the SC fate. This study uses triphenylphosphonium-functionalized gold nanoparticles (TPP-AuNPs) to explore the interplay of intracellular electromagnetic (EM) exposure and the SC fate. Localized EM waves are generated inside neural stem cells (NSCs) to stimulate TPP-AuNPs (AuNPs), targeting the mitochondria through inducing reactive oxygen species and differentiating these cells into neurons. Following laser irradiation of TPP-AuNPs-transfected NSCs, their differentiation to neurons is monitored by tracing the relevant markers both at the genetic and protein levels. The electrophysiology technique is further used to examine the functionality of neurons. The results confirm that TPP-AuNPs subjected to electromotive forces have the potential to regulate cellular fate, although further investigations are still required to shed light on the mechanisms underlying the interaction of EM-stimulated TPP-AuNPs on cellular fate to design highly adjustable cell differentiation and reprogramming methods.

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Antioxidants successfully reduce ROS production in propionic acidemia fibroblasts

Cited by 39 publications

References 28 publications

Propionyl-CoA carboxylase – A review

Propionyl-CoA carboxylase – A review

Sirtuin activation as a therapeutic approach against inborn errors of metabolism

Nanoscale Optoregulation of Neural Stem Cell Differentiation by Intracellular Alteration of Redox Balance

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