Abstract:
:India, resembling other developing nations, is confronting a hastening demographic switch to non-communicable diseases. Inborn errors of metabolism (IEM) constitute a varied heterogeneous group of disorders with variable clinical appearance, primarily in the pediatric populace. Congenital deformities and genetic disorders are significant for mortality throughout the world, and the Indian scenario is not very different. IEMs are a group of monogenic issues described by dysregulation of the metabolic networks that bring about development and homeostasis. Incipient evidence focuses on oxidative stress and mitochondrial dysfunction as significant contributors to the multiorgan modifications are detected in a few IEMs. The amassing of toxic metabolites in organic acidurias, respiratory chain, and fatty acid oxidation ailments inhibit mitochondrial enzymes and processes, bringing about elevated levels of reactive oxygen species (ROS). In different IEMs, as in homocystinuria, various sources of ROS have been suggested. In patients' samples along with cellular and experimental animal models, a few investigations have recognized substantial increments in ROS levels alongside diminishes in antioxidant defenses, relating with oxidative damage to proteins, lipids as well as DNA. Elevated ROS levels interrupt redox signaling pathways controlling biological processes such as cell development, differentiation, or apoptosis; however, few investigations explore these processes in IEMs. This review depicts the mitochondrial dysfunction, oxidative stress, redox signaling in branched-chain amino acid disorders, further organic acidurias, and homocystinuria, alongside the latest research investigating the proficiency of antioxidants in addition to mitochondria-targeted therapies as therapeutic components in these diseases.
journal_name
Free Radic Resjournal_title
Free radical researchauthors
Ray SK,Mukherjee Sdoi
10.1080/10715762.2021.1877286subject
Has Abstractpub_date
2021-01-27 00:00:00pages
1-14eissn
1071-5762issn
1029-2470pub_type
杂志文章abstract::The effects of intravenous administration of alpha-phenyl N-tert-butyl nitrone (PBN) on cortical cerebral blood flow (CBF) were examined in Wistar rats under pentobarbital anesthesia and artificial ventilation. The cortical CBF in parietal cortex was measured by laser Doppler flowmetry. Intravenous administrations of ...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.3109/10715769509064017
更新日期:1995-07-01 00:00:00
abstract::Brain ischemia and the following reperfusion are important causes for brain damage and leading causes of brain morbidity and human mortality. Numerous observations exist describing the neuronal damage during ischemia/reperfusion, but the outcome of such conditions towards glial cells still remains to be elucidated. Mi...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715760600978807
更新日期:2007-02-01 00:00:00
abstract::Previous studies have shown that glucose-6-phosphate dehydrogenase (G6PD)-deficient cells are under increased oxidative stress and undergo premature cellular senescence. The present study demonstrates that G6PD-deficient cells cultured under 3% oxygen concentration had an extended replicative lifespan, as compared wit...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715760601184819
更新日期:2007-05-01 00:00:00
abstract::γ-Tocopherol (γ-T) scavenges reactive nitrogen species (RNS) to form 5-NO2-γ-T (NGT). However, α-T supplementation decreases circulating γ-T, which could limit its RNS scavenging activities. We hypothesized that α-T supplementation would mitigate NGT accumulation by impairing γ-T status. Healthy smokers (21 ± 1 y, n =...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.3109/10715762.2015.1040788
更新日期:2015-01-01 00:00:00
abstract::In addition to insulin, glycemic control involves thyroid hormones. However, an excess of thyroid hormone can disturb the blood glucose equilibrium, leading to alterations of carbohydrate metabolism and, eventually, diabetes. Indeed, experimental and clinical hyperthyroidism is often accompanied by abnormal glucose to...
journal_title:Free radical research
pub_type: 杂志文章,评审
doi:10.1080/10715762.2019.1590567
更新日期:2019-03-01 00:00:00
abstract::Carotenoids scavenge simple lipid-like alkylperoxyl radicals. However, the rate constant is too low to be determined directly and the mechanism is likewise not known with certainty [Mortensen, A. and Skibsted, L.H. (1998) FEBS Lett. 426, 392-396]. It is demonstrated that carotenoids react with peroxyl radicals only sl...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715760290006501
更新日期:2002-02-01 00:00:00
abstract::To define the molecular mechanism(s) of carvedilol inhibition of lipid peroxidation we have utilized model systems that allow us to study the different reactions involved in this complex process. Carvedilol inhibits the peroxidation of sonicated phosphatidylcholine liposomes triggered by FeCl2 addition whereas atenolo...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715769800300421
更新日期:1998-11-01 00:00:00
abstract::Many epidemiological data indicate the presence of a strong familial component of longevity that is largely determined by genetics, and a number of possible associations between longevity and allelic variants of genes have been described. A breakthrough strategy to get insight into the genetics of longevity is the stu...
journal_title:Free radical research
pub_type: 杂志文章,评审
doi:10.1080/10715760600917136
更新日期:2006-12-01 00:00:00
abstract::Reactive oxygen species (ROS) are involved in the mechanism of photoaging and carcinogenesis. Skin is endowed with antioxidant enzymes including superoxide dismutases (SOD): cytosolic copper zinc SOD and mitochondrial manganese SOD. The aim of our study was to estimate the protective effect of manganese against oxidat...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.3109/10715769509065255
更新日期:1995-10-01 00:00:00
abstract::Ionizing radiations such as X-ray and γ-ray can directly or indirectly produce clustered or multiple damages in DNA. Previous studies have reported that overexpression of DNA glycosylases in Escherichia coli (E. coli) and human lymphoblast cells caused increased sensitivity to γ-ray and X-ray irradiation. However, the...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.3109/10715762.2015.1061186
更新日期:2015-10-01 00:00:00
abstract::A study of peroxyl radical-mediated bovine serum albumin oxidation in the presence of the quinolinic aminoxyl 1,2-dihydro-2,2-diphenyl-4-ethoxy-quinoline-1-oxyl (QAO) was carried out in order to test its efficiency as a protein antioxidant. Albumin oxidation was induced by the tert-butylhydroperoxide/PbO2 system. The ...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.3109/10715769409056583
更新日期:1994-10-01 00:00:00
abstract::Radicals from one-electron oxidation of 4-hydroxycinnamate, ferulate and 3,4-dihydroxycinnamate have been formed by reaction with the oxidising triplet state of duroquinone. All three compounds react with triplet duroquinone with second order rate constants close to the diffusion-controlled limit. The identity of the ...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715760100300621
更新日期:2001-07-01 00:00:00
abstract::Testing of pregnant women reveals weakening of neutrophil-mediated effector functions, such as reactive oxygen species generation. This study provides data confirming the phenomenon, gained through application of the flow cytometry technique. Key factors influencing neutrophil functional activity in blood plasma of pr...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715762.2016.1197395
更新日期:2016-08-01 00:00:00
abstract::Autophagy modulation is a potential therapeutic strategy for breast cancer, and a previous study indicated that metformin exhibits significant anti-carcinogenic activity. However, the ability of metformin to induce autophagy and its role in breast cancer cell death remains unclear. In this study, we exposed MCF-7 cell...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715762.2018.1485075
更新日期:2018-08-01 00:00:00
abstract::Effects of 12 wk exercise training on oxidative stress were examined in elderly humans. We measured oxidative stress during a 45 min cycling test by using antipyrine hydroxylation products. Antipyrine breakdown is independent of blood flow to the liver, which is important during exercise. Furthermore, antipyrine react...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715760100300951
更新日期:2001-10-01 00:00:00
abstract::This work describes the synthesis and characterisation of some novel hybrid molecules which contains in the same molecule a free radical moiety of hydrazyl type and a spin-trap moiety of nitrone type. The new compounds synthesized have multiple and easy to follow spectroscopic properties, making them useful as sensors...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715760500385699
更新日期:2006-01-01 00:00:00
abstract::N-nitroso species have recently been detected in animal tissues. Protein N-nitrosotryptophan is the best candidate for this N-nitroso pool. N-nitrosation of N-blocked trytophan derivatives like melatonin (MelH) by N2O3 or peroxynitrite (ONOOH/ONOO- ) has been observed under conditions of pH and reagent concentrations ...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715760600693414
更新日期:2006-09-01 00:00:00
abstract::Tumour necrosis factor alpha (TNF alpha) cytotoxicity is mediated, at least in part, by oxidative stress and phospholipase A2 activation. The first post-receptor events to be observed in TNF alpha-sensitive lines are the generation of superoxide anion (O2-) within the mitochondria and the activation of phospholipase A...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.3109/10715769609149917
更新日期:1996-08-01 00:00:00
abstract::Hispidulin (6-methoxy-5,7,4'-trihydroxyflavone) and eupafolin (6-methoxy-5,7,3',4'-tetrahydroxyflavone), are flavonoids found in the leaves of Eupatorium litoralle. They have recognized antioxidant and antineoplastic properties, although their action mechanisms have not been previously described. We now report the eff...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/13561820500177659
更新日期:2005-12-01 00:00:00
abstract:OBJECTIVES:The aim of the present study was to investigate the potential importance of oxidative stress, measured by isoprostanes-related compounds, as non-traditional risk factor for cardiovascular disease. We planned to examine the relationship between concentrations of plasma F₂-isoprostanes (F₂-IsoPs), isofurans (I...
journal_title:Free radical research
pub_type: 杂志文章,随机对照试验
doi:10.3109/10715762.2012.702900
更新日期:2012-10-01 00:00:00
abstract::Intracellular levels of H2O2 in BHK-21 cells are not static but decline progressively with cell growth. Exposure of cells to inhibitors of catalase, or glutathione peroxidase, not only diminishes this decline but also depresses rates of cell proliferation, suggesting important growth regulatory roles for those antioxi...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.3109/10715769509065268
更新日期:1995-11-01 00:00:00
abstract::Expression of multiple drug resistant (MDR) phenotype and over-expression of P-glycoprotein (P-gp) in the human hepatocellular carcinoma (HCC) cell clone P1(0.5), derived from the PLC/PRF/5 cell line (P5), are associated with strong resistance to oxidative stress and a significant (p < 0.01) increase in intracellular ...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715760410001705140
更新日期:2004-07-01 00:00:00
abstract::Paraquat is a synthetic bipyridylium salt widely used as herbicide and defoliant. Enzyme-catalyzed redoxcycling of paraquat generates oxygen radicals. The toxic, even lethal, effects of paraquat are due to free radical-mediated tissue injury. Ocular lesions, sometimes quite severe, have been observed following acciden...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.3109/10715769509064020
更新日期:1995-07-01 00:00:00
abstract::Chronic obstructive pulmonary disease (COPD) is a lung disease caused by an inflammatory response to various inhaled toxins, especially cigarette smoke. Reactive oxygen species (ROS) and epigenetic abnormality are intimately related to the pathology of COPD, and the overproduction of ROS results in a decrease of histo...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715762.2019.1696965
更新日期:2019-12-06 00:00:00
abstract::The seasonal variation of CuCl2-mediated low density lipoprotein (LDL) oxidation (10 microM Cu2+, lag phase, rate of oxidation and maximum absorbance at 234 nm) were measured in 43 men and women on 4-6 occasions (mean 5.7 +/- 0.5) over a 12-month period. The lag phase averaged 52.7 +/- 0.6 min and did not differ by ge...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/1071576031000061020
更新日期:2003-03-01 00:00:00
abstract::The turning point between apoptosis and necrosis induced by hydrogen peroxide (H2O2) have been investigated using human T-lymphoma Jurkat cells. Cells treated with 50 microM H2O2 exhibited caspase-9 and caspase-3 activation, finally leading to apoptotic cell death. Treatment with 500 microM H2O2 did not exhibit caspas...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715760600632552
更新日期:2006-06-01 00:00:00
abstract::Penicillins, as bactericidal antibiotics, have been widely used to treat infections for several decades. Their structure contains both aromatic and thioether moieties susceptible to free radical oxidation. The (•)OH induced oxidation mechanism of amoxicillin was investigated by pulse radiolysis techniques and by final...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.3109/10715762.2015.1100729
更新日期:2016-01-01 00:00:00
abstract::Heme oxygenase is the rate limiting enzyme in heme degradation to carbon monoxide (CO), iron and bilirubin. The inducible isoform of the protein, heme oxygenase-1 (HO-1), is susceptible to up-regulation by a diverse variety of conditions and agents in mammalian tissue, leading to the common conception that HO-1 is a s...
journal_title:Free radical research
pub_type: 杂志文章,评审
doi:10.1080/10715769900301031
更新日期:1999-12-01 00:00:00
abstract::The in vitro toxicity of the reactive oxygen species generating enzyme xanthine oxidoreductase (XOR) to human peripheral blood lymphocytes was studied after stimulation with phytohaemoagglutinin or anti-CD3/CD28 antibodies. Apoptosis and necrosis were induced by the XOR/hypoxanthine system in a time- and concentration...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.1080/10715760100301191
更新日期:2001-12-01 00:00:00
abstract::Antioxidants are important species which possess the ability to protect the body from damage caused by free radical-induced oxidative stress. A variety of free radical-scavenging antioxidants exist within the body many of which are derived from dietary sources. There is currently much interest in the antioxidant role ...
journal_title:Free radical research
pub_type: 杂志文章
doi:10.3109/10715769709097807
更新日期:1997-03-01 00:00:00