Abstract：
:Fanconi Anemia (FA) is a disease characterized by bone marrow (BM) failure and aplastic anemia. In addition to a defective DNA repair system, other mechanisms are involved in its pathogenesis, such as defective mitochondrial metabolism, accumulation of lipids, and increment of oxidative stress production. To better understand the role of these metabolic alterations in the process of HSC maturation in FA, we evaluated several biochemical and cellular parameters on mononuclear cells isolated from the bone marrow of FA patients or healthy donors. To mimic the cellular residence in the BM niche or their exit from the BM niche to the bloodstream, cells have been grown in hypoxic or normoxic conditions, respectively. The data show that, in normoxic conditions, a switch from anaerobic to aerobic metabolism occurs both in healthy and in pathological samples. However, in FA cells this change is associated with altered oxidative phosphorylation, the increment of oxidative stress production, no activation of the endogenous antioxidant defenses and arrest in the G2M phase of the cell cycle. By contrast, FA cells grown in hypoxic conditions do not show cell cycle and metabolic alterations in comparison to the healthy control, maintaining both an anaerobic flux. The data reported herein suggests that the passage from the BM niche to the bloodstream represents a crucial point in the FA pathogenesis associated with mitochondrial dysfunction.
journal_name
Redox Bioljournal_title
Redox biologyauthors
Cappelli E,Degan P,Bruno S,Pierri F,Miano M,Raggi F,Farruggia P,Mecucci C,Crescenzi B,Naim V,Dufour C,Ravera Sdoi
10.1016/j.redox.2020.101618subject
Has Abstractpub_date
2020-09-01 00:00:00pages
101618issn
2213-2317pii
S2213-2317(20)30823-5journal_volume
36pub_type
杂志文章相关文献
Redox Biology文献大全abstract：:Increased permeability of the intestinal barrier is proposed as an underlying factor for obesity-associated pathologies. Consumption of high fat diets (HFD) is associated with increased intestinal permeabilization and increased paracellular transport of endotoxins which can promote steatosis and insulin resistance. Th...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2017.11.002
更新日期：2018-04-01 00:00:00
abstract：:Ultraviolet light is the dominant environmental oxidative skin stressor and a major skin aging factor. We studied which oxidized phospholipid (OxPL) mediators would be generated in primary human keratinocytes (KC) upon exposure to ultraviolet A light (UVA) and investigated the contribution of OxPL to UVA responses. Ma...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2018.11.006
更新日期：2019-01-01 00:00:00
abstract：:Isolated hepatocytes from young (4-6mo) and old (24-26mo) F344 rats were exposed to increasing concentrations of menadione, a vitamin K derivative and redox cycling agent, to determine whether the age-related decline in Nrf2-mediated detoxification defenses resulted in heightened susceptibility to xenobiotic insult. A...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2016.09.010
更新日期：2016-12-01 00:00:00
abstract：:Cardiovascular disease is the leading cause of the death for both men and women. Although baseline heart physiology and the response to disease are known to differ by sex, little is known about sex differences in baseline molecular signaling, especially with regard to redox biology. In this review, we describe current...
journal_title：Redox biology
pub_type： 杂志文章,评审
doi：10.1016/j.redox.2020.101441
更新日期：2020-04-01 00:00:00
abstract：:Keloids exhibit metabolic reprogramming including enhanced glycolysis and attenuated oxidative phosphorylation. Hypoxia induces a series of protective responses in mammalian cells. However, the metabolic phenotype of keloid fibroblasts under hypoxic conditions remains to be elucidated. The present study aimed to inves...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2020.101815
更新日期：2021-01-01 00:00:00
abstract：:Mouse hindlimb ischemia has been widely used as a model to study peripheral artery disease. Genetic modulation of the enzymatic source of oxidants or components of the antioxidant system reveal that physiological levels of oxidants are essential to promote the process of arteriogenesis and angiogenesis after femoral a...
journal_title：Redox biology
pub_type： 杂志文章,评审
doi：10.1016/j.redox.2017.04.040
更新日期：2017-08-01 00:00:00
abstract：:The nuclear protein HMGB1 (high mobility group box 1) is secreted by monocytes-macrophages in response to inflammatory stimuli and serves as a danger-associated molecular pattern. Acetylation and phosphorylation of HMGB1 are implicated in the regulation of its nucleocytoplasmic translocation for secretion, although in...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2019.101203
更新日期：2019-06-01 00:00:00
abstract：:Mitochondria are critical sources of hydrogen peroxide (H2O2), an important secondary messenger in mammalian cells. Recent work has shown that O2•-/H2O2 emission from individual sites of production in mitochondria is regulated by protein S-glutathionylation. Here, we conducted the first examination of O2•-/H2O2 releas...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2017.12.006
更新日期：2018-05-01 00:00:00
abstract：:Chronic alcoholism disrupts mitochondrial function and often results in alcoholic cardiomyopathy (ACM). Fas-activated serine/threonine kinase (FASTK) is newly recognized as a key post-transcriptional regulator of mitochondrial gene expression. However, the modulatory role of FASTK in cardiovascular pathophysiology rem...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2020.101778
更新日期：2021-01-01 00:00:00
abstract：BACKGROUND:Vitamin D status [25(OH)D] has recently been reported to be associated with altered cellular bioenergetic profiles of peripheral blood mononuclear cells (PBMCs). No study has tracked the seasonal variation of 25(OH)D and its putative influence on whole body energy metabolism, cellular bioenergetic profiles, ...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2017.04.009
更新日期：2017-08-01 00:00:00
abstract：:Oxidative stress plays a major part in the pathogenesis of drug-induced liver injury. Yet, overcoming it with other xenobiotics impose additional risks. In this study, we consider the use of natural-occurring and purified Vitamin E analogs as hepatoprotective agents. Vitamin E is well-known for its intrinsic antioxida...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2015.01.013
更新日期：2015-01-01 00:00:00
abstract：:Despite extensive research that has been carried out over the past three decades in the field of renal ischaemia-reperfusion (I/R) injury, the pathogenic role of mitochondrial fission in renal I/R injury is poorly understood. The aim of our study is to investigate the molecular mechanism by which mammalian STE20-like ...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2018.10.012
更新日期：2019-01-01 00:00:00
abstract：:Gallbladder cancer (GBC) is a highly malignant bile duct cancer with poor prognosis characterized by its insensitivity to chemotherapy. Emerging evidence indicates that cytoprotective antioxidation is involved in drug resistance of various cancers; however, the underlying molecular mechanisms remain obscure. Here, we ...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2019.101149
更新日期：2019-04-01 00:00:00
abstract：:In this paper, we describe an assay to analyze simultaneously the oxygen consumption rate (OCR) and superoxide production in a biological system. The analytical set-up uses electron paramagnetic resonance (EPR) spectroscopy with two different isotopically-labelled sensors: 15N-PDT (4-oxo-2,2,6,6-tetramethylpiperidine-...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2020.101852
更新日期：2020-12-28 00:00:00
abstract：:NADPH oxidase 5 (NOX5) is a transmembrane signaling enzyme that produces superoxide in response to elevated cytosolic calcium. In addition to its association with numerous human diseases, NOX5 has recently been discovered to play crucial roles in the immune response and cardiovascular system. Details of NOX5 maturatio...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2020.101656
更新日期：2020-09-01 00:00:00
abstract：:Oxidative stress response in bacteria is mediated through coordination between the regulators of oxidant-remediation systems (e.g. OxyR, SoxR) and nucleoid condensation (e.g. Dps, Fis). However, these genetic factors are either absent or rendered non-functional in the human pathogen Mycobacterium tuberculosis (Mtb). T...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2018.08.006
更新日期：2018-10-01 00:00:00
abstract：:Yeast Sir2 is an NAD-dependent histone deacetylase related to oxidative stress and aging. In a previous study, we showed that Sir2 is regulated by S-glutathionylation of key cysteine residues located at the catalytic domain. Mutation of these residues results in strains with increased resistance to disulfide stress. I...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2019.101229
更新日期：2019-06-01 00:00:00
abstract：:Artesunate (ART) is a prominent anti-malarial with significant anti-cancer properties. Our previous studies showed that ART enhances lysosomal function and ferritin degradation, which was necessary for its anti-cancer properties. ART targeting to mitochondria also significantly improved its efficacy, but the effect of...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2018.07.025
更新日期：2018-10-01 00:00:00
abstract：:Transcription factor Nuclear factor erythroid 2-related factor 2 (Nrf2) and its negative regulator, the E3 ligase adaptor Kelch-like ECH-associated protein 1 (Keap1), control the redox and metabolic homeostasis and oxidative stress. Inhibitors of Keap1-Nrf2 interaction are promising in oxidative stress related inflamm...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2020.101565
更新日期：2020-07-01 00:00:00
abstract：:Mitochondria are often regarded as a major source of reactive oxygen species (ROS) in animal cells, with H2O2 being the predominant ROS released from mitochondria; however, it has been recently demonstrated that energized brain mitochondria may act as stabilizers of H2O2 concentration (Starkov et al. [1]) based on the...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2015.05.001
更新日期：2015-08-01 00:00:00
abstract：:Ischemia heart disease is the leading cause of death world-widely and has increased prevalence and exacerbated myocardial infarction with aging. Sestrin2, a stress-inducible protein, declines with aging in the heart and the rescue of Sestrin2 in the aged mouse heart improves the resistance to ischemic insults caused b...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2020.101556
更新日期：2020-07-01 00:00:00
abstract：:NRF2 is a redox-responsive transcription factor that regulates expression of cytoprotective genes via its interaction with DNA sequences known as antioxidant response elements (AREs). NRF2 activity is induced by oxidative stress, but oxidative stress is not the only context in which NRF2 can be activated. Mutations th...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2018.07.026
更新日期：2018-10-01 00:00:00
abstract：:Hypertension is one of the major predisposing factors for neurodegenerative disease characterized with activated renin-angiotensin system (RAS) in both periphery and brain. Vitamin D (VitD) is recently recognized as a pleiotropic hormone with strong neuroprotective properties. While multiple lines of evidence suggest ...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2019.101295
更新日期：2019-09-01 00:00:00
abstract：:Muscle mass and strength are very important for exercise performance. Training-induced musculoskeletal injuries usually require periods of complete immobilization to prevent any muscle contraction of the affected muscle groups. Disuse muscle wasting will likely affect every sport practitioner in his or her lifetime. E...
journal_title：Redox biology
pub_type： 杂志文章,评审
doi：10.1016/j.redox.2020.101531
更新日期：2020-08-01 00:00:00
abstract：:The inducible form of Heme Oxygenase-1 (HO-1), a major endoplasmic reticulum (ER) associated heme protein, is known to play important roles in protection against oxidative and chemical stress by degrading free heme released from degradation of heme proteins. In this study we show that induced expression of HO-1 by sub...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2013.07.004
更新日期：2013-07-23 00:00:00
abstract：:Highly active anti-retrovirus therapy (HAART) has been used to block the progression and symptoms of human immunodeficiency virus infection. Although it decreases morbidity and mortality, clinical use of HAART has also been linked to various adverse effects such as severe cardiomyopathy resulting from compromised mito...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2017.06.011
更新日期：2017-10-01 00:00:00
abstract：:Oxidative stress and neuronal apoptosis have been demonstrated to be key features in early brain injury (EBI) after subarachnoid hemorrhage (SAH). Previous studies have indicated that Mas receptor activation initiates an anti-oxidative and anti-apoptotic role in the brain. However, whether Mas activation can attenuate...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2018.09.022
更新日期：2019-01-01 00:00:00
abstract：:Mechanistic/mammalian target of rapamycin (mTOR) has emerged as a new potential therapeutic target for gastric cancer. Rapamycin and rapamycin analogs are undergoing clinical trials and have produced clinical responses in a subgroup of cancer patients. However, monotherapy with rapamycin at safe dosage fails to induce...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2016.09.006
更新日期：2016-12-01 00:00:00
abstract：:Hypoxia, a common element in the tumor environment, leads to Hypoxia-Inducible Factor-1α (HIF-1α) stabilization to modulate cellular metabolism as an adaptive response. In a previous study, we showed that inhibition of the nuclear factor erythroid 2-like-2 (NFE2L2; NRF2), a master regulator of many genes coping with e...
journal_title：Redox biology
pub_type： 杂志文章
doi：10.1016/j.redox.2019.101210
更新日期：2019-06-01 00:00:00
abstract：:Oxidative stress resulting from the accumulation of high levels of reactive oxygen species is a salient feature of, and a well-recognised pathological factor for, diverse pathologies. One common mechanism for oxidative stress damage is via the disruption of intracellular ion homeostasis to induce cell death. TRPM2 is ...
journal_title：Redox biology
pub_type： 杂志文章,评审
doi：10.1016/j.redox.2020.101755
更新日期：2020-10-01 00:00:00
