Advanced oxidation protein products in serum of patients with myotonic disease type I: association with serum γ-glutamyltransferase and disease severity

2005 ◽  
Vol 43 (7) ◽  
Author(s):  
Gabriele Siciliano ◽  
Livia Pasquali ◽  
Anna Rocchi ◽  
Michela Falorni ◽  
Fabio Galluzzi ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Characteristic Curve ◽  
Advanced Oxidation ◽  
Clinical Severity ◽  
Total Serum ◽  
Type I ◽  
Advanced Oxidation Protein Products ◽  
Severity Scores

AbstractSteinert's disease (myotonic dystrophy type 1; MD) is caused by a CTG trinucleotide expansion on 19q13.3. Although the pathogenic mechanism underlying multisystem involvement in MD is still unclear, a role of oxidative stress in this disease has been suggested. We investigated 39 MD patients to assess the plasma concentration of advanced oxidation protein products (AOPPs) and γ-glutamyltransferase (GGT) and related them to clinical severity scores. Plasma AOPP levels (p=0.021), total serum GGT activity (p=0.0005) and GGT activity associated with low-density lipoprotein (p=0.0021) were significantly higher in patients than in controls. There was significant correlation between serum GGT levels and AOPPs (r=0.5831; p=0.0022). A statistically significant increase in serum GGT with age was found in MD patients (p=0.0193). Receiver operating characteristic curve analysis showed that higher AOPP levels were significantly associated with extra-muscular signs of the disease, i.e., cataracts and heart involvement (area under the curve±SE=0.908±0.083), but not with muscular involvement. The concomitant increment in GGT and AOPPs indicates a possible role of oxidative stress in the pathogenesis of MD type 1, while the association of increased AOPP levels with extra-muscular signs of the disease suggests that individual susceptibility to oxidative stress can modulate the extra-muscular phenotype of the disease.

scholarly journals The Role of MicroRNAs in Diabetes-Related Oxidative Stress

2019 ◽  
Vol 20 (21) ◽  
pp. 5423 ◽  
Author(s):  
Mirza Muhammad Fahd Qadir ◽  
Dagmar Klein ◽  
Silvia Álvarez-Cubela ◽  
Juan Domínguez-Bendala ◽  
Ricardo Luis Pastori
Keyword(s):  
Oxidative Stress ◽  
Target Gene ◽  
Cellular Stress ◽  
Cellular Damage ◽  
Oxygen Species ◽  
Non Coding Rnas ◽  
And Oxidative Stress

Cellular stress, combined with dysfunctional, inadequate mitochondrial phosphorylation, produces an excessive amount of reactive oxygen species (ROS) and an increased level of ROS in cells, which leads to oxidation and subsequent cellular damage. Because of its cell damaging action, an association between anomalous ROS production and disease such as Type 1 (T1D) and Type 2 (T2D) diabetes, as well as their complications, has been well established. However, there is a lack of understanding about genome-driven responses to ROS-mediated cellular stress. Over the last decade, multiple studies have suggested a link between oxidative stress and microRNAs (miRNAs). The miRNAs are small non-coding RNAs that mostly suppress expression of the target gene by interaction with its 3’untranslated region (3′UTR). In this paper, we review the recent progress in the field, focusing on the association between miRNAs and oxidative stress during the progression of diabetes.

scholarly journals Type 1 FSHD with 6–10 Repeated Units: Factors Underlying Severity in Index Cases and Disease Penetrance in Their Relatives Attention

2020 ◽  
Vol 21 (6) ◽  
pp. 2221
Author(s):  
Emmanuelle Salort-Campana ◽  
Farzad Fatehi ◽  
Sadia Beloribi-Djefaflia ◽  
Stéphane Roche ◽  
Karine Nguyen ◽  
...  
Keyword(s):  
Disease Severity ◽  
Facioscapulohumeral Muscular Dystrophy ◽  
Clinical Severity ◽  
Cross Sectional ◽  
Repeat Array ◽  
D4z4 Repeat ◽  
Severity Scores ◽  
Group 2 ◽  
Group 1

Molecular defects in type 1 facioscapulohumeral muscular dystrophy (FSHD) are caused by a heterozygous contraction of the D4Z4 repeat array from 1 to 10 repeat units (RUs) on 4q35. This study compared (1) the phenotype and severity of FSHD1 between patients carrying 6–8 vs. 9–10 RUs, (2) the amount of methylation in different D4Z4 regions between patients with FSHD1 with different clinical severity scores (CSS). This cross-sectional multicenter study was conducted to measure functional scales and for genetic analysis. Patients were classified into two categories according to RUs: Group 1, 6–8; Group 2, 9–10. Methylation analysis was performed in 27 patients. A total of 99 carriers of a contracted D4Z4 array were examined. No significant correlations between RUs and CSS (r = 0.04, p = 0.73) and any of the clinical outcome scales were observed between the two groups. Hypomethylation was significantly more pronounced in patients with high CSS (>3.5) than those with low CSS (<1.5) (in DR1 and 5P), indicating that the extent of hypomethylation might modulate disease severity. In Group 1, the disease severity is not strongly correlated with the allele size and is mostly correlated with the methylation of D4Z4 regions.

Oxidative Stress Is Increased in Chronic Idiopathic Myelofibrosis (CIMF) Patients, and Is Associated with High Levels of Homocysteine (Hcy) and Depletion of Holotranscobalamin (holoTC).

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3618-3618
Author(s):  
Nicola S. Fracchiolla ◽  
Fabrizia Bamonti Catena ◽  
Claudia Vener ◽  
Rossella Calori ◽  
Cristina Novembrino ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Peripheral Blood ◽  
Cardiovascular Events ◽  
Smoking Habit ◽  
White Blood Cells ◽  
Total Serum ◽  
Oxidative Potential ◽  
Clinical Role ◽  
Clinical Variables

Abstract In patients with myeloproliferative disorders, the increased cellular turnover may lead to hyperhomocysteinemia (HH), caused by depletion of folate and/or cobalamin (VitB12). HH represents a cause of oxidative stress, associated with a number of diseases, among which cardiovascular events play an important role. Recently, holoTC, that consist of VitB12 linked to its transport molecule, transcobalamin, has been proposed as a more reliable marker than the dosage of total serum VitB12 to detect the deficiency of this vitamin. The aim of the study was to investigate the clinical role of oxidative stress in CIMF. We performed in 67 CIMF patients (M/F: 38/29; median age 63, range 34–79), the dosage of reactive oxygen species (ROS), total antioxidant capacity (TAC), Hcy, VitB12, folates and holoTC, and correlated the results with the following clinical variables: age, sex, cytogenetics, white blood cells and platelets counts, hemoglobin and LDH levels, splenomegaly and hepatomegaly, hypertension, diabetes, smoking habit, cardiovascular events, secondary tumors, and presence of blasts in peripheral blood. Serum concentrations of VitB12, folates and holoTC were determined by AxSYM B12 or Folate microparticle enzyme immunoassay (MEIA) and new Axis-Shield HoloTC MEIA, respectively; plasma Hcy levels were measured by fluorescence polarization immunoassay (FPIA). All the panel was performed on the Abbott AxSYM analyser. CIMF patients presented increased oxidative stress (lower levels of TAC and higher levels of ROS), compared to a panel of 53 healthy volunteers (Mann Whitney U test, p<0.0001). Higher values of Hcy were detected in the group of CIMF presenting holoTC deficiency (Mann Whitney U test, p=0.03). Consistently, holoTC directly correlated with TAC (Spearman correlation, p=0.01). Interestingly, levels of holoTC and TAC were significantly lower in the group of CIMF patients with blasts in the peripheral blood (Mann Whitney U test, p=0.01 and p=0.009, respectively). Notably, levels of VitB12 and folates did not correlate with Hcy, holoTC, TAC or ROS. No correlations were found between the levels of Hcy, holoTC, TAC, ROS, VitB12 or folates and the remaining clinical variables considered. In conclusion, we demonstrated that CIMF patients present an increase in oxidative stress compared to healthy controls. We observed that holoTC depletion is associated to HH and reduced TAC, consistently with an increased oxidative potential. These findings indicate the presence of a clinically significant VitB12 deficiency, detectable only by holoTC dosage, in the presence of normal levels of total serum VitB12. The other interesting finding is the association of holoTC and TAC deficiency with the presence of blasts in the peripheral blood of the patients, suggesting a role of oxidative damage in CIMF progression. Therefore, our findings suggest that, in CIMF patients, the dosage of ROS, TAC, holoTC, and Hcy, may represent a valuable clinical aid in the diagnosis of unapparent VitB12 deficiency, and provide a rational basis to prevent and/or correct the increase in oxidative potential associated with this disease.

scholarly journals Angiotensin II Removes Kidney Resistance Conferred by Ischemic Preconditioning

2014 ◽  
Vol 2014 ◽  
pp. 1-10 ◽  
Author(s):  
Hee-Seong Jang ◽  
Jee In Kim ◽  
Jinu Kim ◽  
Jeen-Woo Park ◽  
Kwon Moo Park
Keyword(s):  
Oxidative Stress ◽  
Angiotensin Ii ◽  
Ischemic Preconditioning ◽  
Inflammatory Responses ◽  
Ischemia Reperfusion ◽  
Sham Operation ◽  
Kidney Fibrosis ◽  
Losartan Treatment

Ischemic preconditioning (IPC) by ischemia/reperfusion (I/R) renders resistance to the kidney. Strong IPC triggers kidney fibrosis, which is involved in angiotensin II (AngII) and its type 1 receptor (AT1R) signaling. Here, we investigated the role of AngII/AT1R signal pathway in the resistance of IPC kidneys to subsequent I/R injury. IPC of kidneys was generated by 30 minutes of bilateral renal ischemia and 8 days of reperfusion. Sham-operation was performed to generate control (non-IPC) mice. To examine the roles of AngII and AT1R in IPC kidneys to subsequent I/R, IPC kidneys were subjected to either 30 minutes of bilateral kidney ischemia or sham-operation following treatment with AngII, losartan (AT1R blocker), or AngII plus losartan. IPC kidneys showed fibrotic changes, decreased AngII, and increased AT1R expression. I/R dramatically increased plasma creatinine concentrations in non-IPC mice, but not in IPC mice. AngII treatment in IPC mice resulted in enhanced morphological damage, oxidative stress, and inflammatory responses, with functional impairment, whereas losartan treatment reversed these effects. However, AngII treatment in non-IPC mice did not change I/R-induced injury. AngII abolished the resistance of IPC kidneys to subsequent I/R via the enhancement of oxidative stress and inflammatory responses, suggesting that the AngII/AT1R signaling pathway is associated with outcome in injury-experienced kidney.

scholarly journals Role of Estrogens in the Size of Neuronal Somata of Paravaginal Ganglia in Ovariectomized Rabbits

2017 ◽  
Vol 2017 ◽  
pp. 1-12 ◽  
Author(s):  
Laura G. Hernández-Aragón ◽  
Verónica García-Villamar ◽  
María de los Ángeles Carrasco-Ruiz ◽  
Leticia Nicolás-Toledo ◽  
Arturo Ortega ◽  
...  
Keyword(s):  
Estradiol Benzoate ◽  
Total Serum ◽  
Serum Estradiol ◽  
Neuronal Somata ◽  
Satellite Glial Cells ◽  
Neuronal Soma ◽  
Soma Size ◽  
Alpha Type

We aimed to determine the role of estrogens in modulating the size of neuronal somata of paravaginal ganglia. Rabbits were allocated into control (C), ovariectomized (OVX), and OVX treated with estradiol benzoate (OVX + EB) groups to evaluate the neuronal soma area; total serum estradiol (E2) and testosterone (T) levels; the percentage of immunoreactive (ir) neurons anti-aromatase, anti-estrogen receptor (ERα, ERβ) and anti-androgen receptor (AR); the intensity of the immunostaining anti-glial cell line-derived neurotrophic factor (GDNF) and the GDNF family receptor alpha type 1 (GFRα1); and the number of satellite glial cells (SGCs) per neuron. There was a decrease in the neuronal soma size for the OVX group, which was associated with low T, high percentages of aromatase-ir and neuritic AR-ir neurons, and a strong immunostaining anti-GDNF and anti-GFRα1. The decrease in the neuronal soma size was prevented by the EB treatment that increased the E2 without affecting the T levels. Moreover, there was a high percentage of neuritic AR-ir neurons, a strong GDNF immunostaining in the SGC, and an increase in the SGCs per neuron. Present findings show that estrogens modulate the soma size of neurons of the paravaginal ganglia, likely involving the participation of the SGC.

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