Oxidative Stress in Down and Williams-Beuren Syndromes: An Overview

Ferrari, Marta; Stagi, Stefano

doi:10.3390/molecules26113139

Cited by 15 publications

(18 citation statements)

References 86 publications

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“…A previous study revealed that DATS preserved cardiac function via decreasing oxidative stress and ER stress-mediated apoptosis ( Yu et al, 2017a ). SOD1 is an important enzyme against oxidative stress ( Ferrari and Stagi, 2021 ), which can be used to evaluate the level of oxidative stress in skin flaps. In the current study, the proteins SOD1, eNOS, and HO-1 that exert antioxidative stress effects and the level of GSH were increased by DATS treatment in flap tissue, indicating that DATS has antioxidant properties for flap survival.…”

Section: Discussionmentioning

confidence: 99%

Diallyl Trisulfide Enhances the Survival of Multiterritory Perforator Skin Flaps

et al. 2022

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The multiterritory perforator flap is one of the widest flap patterns used to repair tissue defects. However, flap necrosis of the distal part is still a challenging issue for plastic surgeons. Diallyl trisulfide (DATS) is an efficient ingredient extracted from garlic, exerting many important effects on different diseases. Our experiment aims to reveal whether DATS has a beneficial effect on the survival of perforator flaps and to explore its mechanism of action. The results showed that DATS enhanced angiogenesis and autophagy and reduced cell apoptosis and oxidative stress, thereby improving the survival rate of skin flaps. After co-administration with autophagy inhibitor 3-methyladenine (3MA), perforator flap survival was further improved. Mechanistically, we showed that PI3K/Akt and AMPK-HIF-1α signaling pathways in flap were activated under DATS treatment. All in all, DATS promoted the survival of multiterritory perforator flaps via the synergistic regulation of PI3K/Akt and AMPK-HIF-1α signaling pathways, and inhibition of DATS-induced autophagy further improves flap survival.

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Section: Discussionmentioning

confidence: 99%

Diallyl Trisulfide Enhances the Survival of Multiterritory Perforator Skin Flaps

et al. 2022

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“…While a variety of diseases are reportedly associated with altered SOD1 activity, the following two hereditary diseases have attracted much attention. Because a trisomy of the 21st chromosome is the cause for Down's syndrome and SOD1 is mapped to the region where the putative causative gene is thought to be present, an elevation in SOD1 activity has been the suspected cause of Down syndrome [58]. Pathological abnormalities in the neuromuscular junctions in the tongue are characteristically observed in transgenic mice overexpressing SOD1 [59].…”

Section: Sod1mentioning

confidence: 99%

Superoxide Radicals in the Execution of Cell Death

2022

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Superoxide is a primary oxygen radical that is produced when an oxygen molecule receives one electron. Superoxide dismutase (SOD) plays a primary role in the cellular defense against an oxidative insult by ROS. However, the resulting hydrogen peroxide is still reactive and, in the presence of free ferrous iron, may produce hydroxyl radicals and exacerbate diseases. Polyunsaturated fatty acids are the preferred target of hydroxyl radicals. Ferroptosis, a type of necrotic cell death induced by lipid peroxides in the presence of free iron, has attracted considerable interest because of its role in the pathogenesis of many diseases. Radical electrons, namely those released from mitochondrial electron transfer complexes, and those produced by enzymatic reactions, such as lipoxygenases, appear to cause lipid peroxidation. While GPX4 is the most potent anti-ferroptotic enzyme that is known to reduce lipid peroxides to alcohols, other antioxidative enzymes are also indirectly involved in protection against ferroptosis. Moreover, several low molecular weight compounds that include α-tocopherol, ascorbate, and nitric oxide also efficiently neutralize radical electrons, thereby suppressing ferroptosis. The removal of radical electrons in the early stages is of primary importance in protecting against ferroptosis and other diseases that are related to oxidative stress.

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“…The etiology of this disease is associated with the induction of oxidative stress. The main contributing genes, including superoxide dismutase-1 and neutrophil cytosolic factor-1, are primarily involved in the regulation of the redox-state ( 5 - 7 ). WBSCR22 is localized on chromosome 7 (7q11.23) and constitutes an S-adenosyl-L-methionine (SAM) motif and nuclear localization signal with well-preserved features of methyltransferase family proteins ( 8 , 9 ).…”

Section: Introductionmentioning

confidence: 99%

WBSCR22 and TRMT112 synergistically suppress cell proliferation, invasion and tumorigenesis in pancreatic cancer via transcriptional regulation of ISG15

et al. 2022

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Pancreatic cancer (PC) is one of the most aggressive and devastating types of cancer owing to its poor prognosis and deadly characteristics. It is well established that aberrations in the expression of key regulatory genes, namely tumor suppressors and oncogenes, predispose patients to progression and metastasis of PC. Upregulation of Williams-Beuren syndrome chromosomal region 22 (WBSCR22) expression, a ribosomal biogenesis factor, has been reported in multiple types of human cancer. However, the role of WBSCR22 and its underlying mechanism in PC have not been well investigated. In the present study, the tumor suppressive role of WBSCR22 was reported in PC for the first time; the results indicated that WBSCR22 overexpression (OE) significantly suppressed cellular proliferation, migration, invasion and tumorigenesis in vivo and in vitro. RNA-sequencing analysis revealed that WBSCR22 negatively regulated the transcription of interferon-stimulated gene 15 (ISG15) downstream, which is a ubiquitin-like modifier protein involved in metabolic and proteasome degradation pathways, while the antitumor function of WBSCR22-OE could be rescued by ISG15 OE. In addition, the oncogenic role of ISG15 was further confirmed in PC; its upregulation promoted the proliferation, migration, invasion and tumorigenesis of PC. Furthermore, WBSCR22 and its cofactor tRNA methyltransferase activator subunit 11-2 (TRMT112) functioned synergistically in PC, and concurrent ectopic OE of WBSCR22 and TRMT112 further promoted the tumor suppressive potential of WBSCR22 in PC. Collectively, the findings indicated that WBSCR22 played an important role in PC development and that the WBSCR22/ISG15 axis may provide a novel therapeutic strategy for PC treatment.

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Oxidative Stress in Down and Williams-Beuren Syndromes: An Overview

Cited by 15 publications

References 86 publications

Diallyl Trisulfide Enhances the Survival of Multiterritory Perforator Skin Flaps

Diallyl Trisulfide Enhances the Survival of Multiterritory Perforator Skin Flaps

Superoxide Radicals in the Execution of Cell Death

WBSCR22 and TRMT112 synergistically suppress cell proliferation, invasion and tumorigenesis in pancreatic cancer via transcriptional regulation of ISG15

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