Decoding the muscle transcriptome of patients with late onset Pompe disease reveals markers of disease progression.

Late-onset Pompe Disease (LOPD) is a rare genetic disorder caused by the deficiency of acid alpha-glucosidase leading to progressive cellular dysfunction due to the accumulation of glycogen in the lysosome. The mechanism of relentless muscle damage - a classic manifestation of the disease - has been extensively studied by analysing the whole muscle tissue; however, little, if any, is known about transcriptional heterogeneity among nuclei within the multinucleated skeletal muscle cells. This is the first report of application of single nuclei RNA sequencing to uncover changes in the gene expression profile in muscle biopsies from eight patients with LOPD and four muscle samples from age and gender matched healthy controls. We matched these changes with histology findings using GeoMx Spatial Transcriptomics to compare the transcriptome of control myofibers from healthy individuals with non-vacuolated (histologically unaffected) and vacuolated (histologically affected) myofibers of LODP patients. We observed an increase in the proportion of slow and regenerative muscle fibers and macrophages in LOPD muscles. The expression of the genes involved in glycolysis was reduced, whereas the expression of the genes involved in the metabolism of lipids and amino acids was increased in non-vacuolated fibers, indicating early metabolic abnormalities. Additionally, we detected upregulation of autophagy genes, and downregulation of the genes involved in ribosomal and mitochondrial function leading to defective oxidative phosphorylation. The upregulation of the genes associated with inflammation, apoptosis and muscle regeneration was observed only in vacuolated fibers. Notably, enzyme replacement therapy - the only available therapy for the disease - showed a tendency to restore metabolism dysregulation, particularly within slow fibers. A combination of single nuclei RNA sequencing and spatial transcriptomics revealed the landscape of normal and the diseased muscle, and highlighted the early abnormalities associated with the disease progression. Thus, the application of these two new cutting-edge technologies provided insight into the molecular pathophysiology of muscle damage in LOPD and identified potential avenues for therapeutic intervention.

Effect of air-abrasion pretreatment with three desensitizing agents on efficacy of in-office tooth bleaching.

OBJECTIVE: To evaluate the influence of air-abrasion of enamel with three different desensitizing powders on the whitening effect of a bleaching gel containing 40% H2O2, which was used for in-office tooth bleaching. MATERIALS AND METHODS: Forty human incisors, extracted and prepared, were acquired for this study and subsequently randomized into four groups (n = 10). The control group specimens underwent no pretreatment prior to the bleaching procedure, whereas the remaining three groups underwent air abrasion using distinct desensitizing powders; (a) Sylc, which contains bioglass 45S5; (b) BioMinF, which contains calcium phospho-fluoro-silicate glass; and (c) MI Pearls, which contains nano-hydroxyapatite, 1 h preceding the Opalescence Boost PF 40% bleaching procedure. Color measurements were conducted using a double-beam UV-Vis spectrophotometer at four distinct time points (prior to bleaching, 24 h, 15 days, and 30 days post-bleaching). RESULTS: Tooth color change outcomes revealed that there were no statistically significant results with respect to the interaction of the two criteria (treatments and time) (p = 0.990). Additionally, there were no statistically significant results with respect to the main effects of treatments (p = 0.385), while there were statistically significant effects with respect to the time criterion (p = 0.013). CONCLUSIONS: The use of the tested desensitizing powders prior the bleaching procedure did not affect the tooth color change induced by the tested bleaching agent. CLINICAL SIGNIFICANCE: Tooth color change and whiteness are not affected by air-abrasion desensitizing treatments when applied prior to in-office bleaching procedures.

Transoesophageal echocardiography beyond the echo-laboratory. An expert consensus paper of the working group of echocardiography of the hellenic society of cardiology.

Transoesophageal echocardiography (TOE) is a well-established and valid imaging modality, providing more accurate and of higher quality information than transthoracic echocardiography (TTE) for several specific diagnoses and recently a useful guide of an increasing number of catheter-based and surgical interventions. The present paper represents an effort by the Echocardiography Working Group (WG) of the Hellenic Society of Cardiology to state the essential steps of the TOE exam performed beyond the echo lab: a) in the operating rooms intraoperatively during either transcatheter interventions, or cardiothoracic surgery and b) in the intensive care unit for critically ill patients' monitoring. This paper includes information and tips and tricks about the pre-procedural evaluation, the procedural echocardiographic guidance, and post-procedural evaluation of the result and potential complications.

Myotonia congenita in a Greek cohort: Genotype spectrum and impact of the CLCN1:c.501C > G variant as a genetic modifier.

INTRODUCTION/AIMS: Myotonia congenita (MC) is the most common hereditary channelopathy in humans. Characterized by muscle stiffness, MC may be transmitted as either an autosomal dominant (Thomsen) or a recessive (Becker) disorder. MC is caused by variants in the voltage-gated chloride channel 1 (CLCN1) gene, important for the normal repolarization of the muscle action potential. More than 250 disease-causing variants in the CLCN1 gene have been reported. This study provides an MC genotype-phenotype spectrum in a large cohort of Greek patients and focuses on novel variants and disease epidemiology, including additional insights for the variant CLCN1:c.501C > G. METHODS: Sanger sequencing for the entire coding region of the CLCN1 gene was performed. Targeted segregation analysis of likely candidate variants in additional family members was performed. Variant classification was based on American College of Medical Genetics (ACMG) guidelines. RESULTS: Sixty-one patients from 47 unrelated families were identified, consisting of 51 probands with Becker MC (84%) and 10 with Thomsen MC (16%). Among the different variants detected, 11 were novel and 16 were previously reported. The three most prevalent variants were c.501C > G, c.2680C > T, and c.1649C > G. Additionally, c.501C > G was detected in seven Becker cases in-cis with the c.1649C > G. DISCUSSION: The large number of patients in whom a diagnosis was established allowed the characterization of genotype-phenotype correlations with respect to both previously reported and novel findings. For the c.501C > G (p.Phe167Leu) variant a likely nonpathogenic property is suggested, as it only seems to act as an aggravating modifying factor in cases in which a pathogenic variant triggers phenotypic expression.

Practical guidance and clinical applications of transoesophageal echocardiography. A position paper of the working group of echocardiography of the Hellenic Society of Cardiology.

Transoesophageal echocardiography (TOE) is a well-established imaging modality, providing more accurate and of higher quality information than transthoracic echocardiography (TTE) for a wide spectrum cardiac and extra-cardiac diseases. The present paper represents an effort by the Echocardiography Working Group (WG) of the Hellenic Cardiology Society to state the essential steps of the typical TOE exam performed in echo lab. This is an educational text, describing the minimal requirements and the preparation of a meticulous TOE examination. Most importantly, it gives practical instructions to obtain and optimize TOE views and analyses the implementation of a combined two-and multi-dimensional protocol for the imaging of the most common cardiac structures during a TOE. In the second part of the article a comprehensive review of the contemporary use of TOE in a wide spectrum of valvular and non-valvular cardiac diseases is provided, based on the current guidelines and the experience of the WG members.

Muscle Ultrasound Echo Intensity and Fiber Type Composition in Young Females.

Ultrasonography has been extensively used to evaluate skeletal muscle morphology. The echo intensity, i.e., the mean pixel intensity of a specific region of interest in an ultrasound image, may vary among muscles and individuals with several intramuscular parameters presumed to influence it. The purpose of this study was to investigate the correlation between muscle echo intensity and muscle fiber type composition in humans. Thirteen female physical education students (age: 22.3 ± 5.4 years, height: 1.63 ± 0.06 m, body mass: 59.9 ± 7.4 kg) with no history of systematic athletic training participated in the study. Body composition with dual X-ray absorptiometry, leg-press maximum strength (1-RM), echo intensity, and the cross-sectional area (CSA) of the vastus lateralis (VL) muscle according to ultrasonography were measured. Muscle biopsies were harvested from the VL site where the echo intensity was measured. VL echo intensity was not significantly correlated with the percentage of type I muscle fibers or with the percentage area of type I muscle fibers. However, when VL echo intensity was corrected for the subcutaneous fat thickness at the site of the measurement, it was significantly correlated with the percentage of type I muscle fibers (r = 0.801, p < 0.01) and the percentage area of type I muscle fibers (r = 0.852, p < 0.01). These results suggest that the echo intensity of the vastus lateralis muscle corrected for the subcutaneous fat thickness at the measurement site may provide an estimate of the muscle fiber type composition, at least in young moderately trained females.

Recovery during Successive 120-min Football Games: Results from the 120-min Placebo/Carbohydrate Randomized Controlled Trial.

PURPOSE: This study aimed to examine the recovery kinetics (i.e., time-dependent changes) of performance-related variables between two 120-min male football games performed 3 d apart with and without carbohydrate supplementation. METHODS: Twenty male players (20 ± 1 yr; body fat, 14.9% ± 5.1%; maximal oxygen consumption, 59.4 ± 3.7 mL·kg -1 ·min -1 ) participated in two 120-min football games (G1, G2) according to a randomized, two-trial, repeated-measures, crossover, double-blind design. Participants received carbohydrate/placebo supplements during recovery between games. Field activity was monitored during the games. Performance testing and blood sampling were performed before and at 90 and 120 min of each game. Muscle biopsies were collected at baseline and at 90 and 120 min of G1 and pre-G2. RESULTS: Compared with G1, G2 was associated with reduced total distance (10,870 vs 10,685 m during 90 min and 3327 vs 3089 m during extra 30 min; P = 0.007-0.038), average (6.7 vs 6.2 km/h during extra 30-min game-play; P = 0.007) and maximal speed (32.2 vs 30.2 km/h during 90 min and 29.0 vs 27.9 km/h during extra 30 min; P < 0.05), accelerations/decelerations ( P < 0.05), and mean heart rate ( P < 0.05). Repeated sprint ability ( P < 0.001), jumping ( P < 0.05), and strength ( P < 0.001) performance were compromised before and during G2. Muscle glycogen was not restored at G2 baseline ( P = 0.005). Extended game-play reduced lymphocyte, erythrocyte counts, hematocrit, hemoglobin, reduced glutathione ( P < 0.05) and increased delayed onset of muscle soreness, creatine kinase activity, blood glycerol, ammonia, and protein carbonyls ( P < 0.05) before and during G2. Pax7 + ( P = 0.004) and MyoD + cells ( P = 0.019) increased at baseline G2. Carbohydrate supplementation restored performance and glycogen, reduced glycerol and delayed onset of muscle soreness responses, and increased leukocyte counts and Pax7 + and MyoD + cells. CONCLUSIONS: Results suggest that extended football games induce a prolonged recovery of performance, which may be facilitated by carbohydrate supplementation during a congested game fixture.

The diagnostic value of stress echocardiography with limited myocardial ischemia in high-risk patients.

BACKGROUND: The diagnostic value of limited myocardial ischemia in DSE is not well known. OBJECTIVES: We investigated whether myocardial ischemia during dobutamine stress echocardiography (DSE) in 1 apical segment of any of the ventricular walls of the left ventricle relates to the anatomical and functional stenosis of the suppling coronary artery. METHODS: Our observational, prospective study enrolled 212 patients, symptomatic or asymptomatic, with newly diagnosed limited myocardial ischemia on DSE. Almost 25% of them had already known CAD, while the rest were divided into low-risk and high-risk groups, integrating 1-2 and ≥3 classical cardiovascular risk factors, respectively. After DSE, all patients underwent invasive coronary angiography (ICA) and were followed up for one year. In coronary arteries distributing ischemic area, the calculated stenosis ≥50% and FFR<0.8 were considered anatomically and functionally significant, respectively. In the latter cases, the patients underwent coronary revascularization. RESULTS: Significant anatomical and functional stenosis of the supplying coronary artery was common among patients with already known CAD (62.5% and 44.5%, respectively) or those without CAD but a high-risk profile (60.2% and 25.6%, respectively). In logistic regression analysis, CAD revascularization was independently determined by an already known CAD, diabetes mellitus, and high-risk profile. During follow-up, 24 patients experienced ACS or new angina episodes, which were associated with diabetes and smoking in univariate analysis. CONCLUSION: Limited myocardial ischemia may implicate significant anatomical and functional coronary stenosis among individuals with a history of CAD or those without known CAD but a high-risk profile. The prognostic value of our findings requires further investigation.

Exercise and cardiac rehabilitation in hypertensive patients with heart failure with preserved ejection fraction: A position statement on behalf of the Working Group of Arterial Hypertension of the Hellenic Society of Cardiology.

Arterial hypertension is a major cause of cardiovascular morbidity and mortality and the most common cause of comorbidity in heart failure (HF) with preserved ejection fraction (HFpEF). As an adjunct to medication, healthy lifestyle modifications with emphasis on regular exercise are strongly recommended by both the hypertension and the HF guidelines of the European Society of Cardiology. Several long-term studies have shown that exercise is associated with a reduction in all-cause mortality, a favorable cardiac and metabolic risk profile, mental health, and other non-cardiovascular benefits, as well as an improvement in overall quality of life. However, the instructions for the prescriptive or recommended exercise in hypertensive patients and, more specifically, in those with HFpEF are not well defined. Moreover, the evidence is based on observational or small randomized studies, while well-designed clinical trials are lacking. Despite the proven benefit and the guidelines' recommendations, exercise programs and cardiac rehabilitation in patients with hypertensive heart disease and HFpEF are grossly underutilized. This position statement provides a general framework for exercise and exercise-based rehabilitation in patients with hypertension and HFpEF, guides clinicians' rehabilitation strategies, and facilitates clinical practice. It has been endorsed by the Working Group of Arterial Hypertension of the Hellenic Society of Cardiology and is focused on the Health Care System in Greece.

Long-term outcomes, residual symptoms and quality of life in COVID-19 hospitalized patients: A 12-month longitudinal study.

The long-term impact of the coronavirus disease 2019 (COVID-19) pandemic is a critical public health concern. The presence of residual symptoms in COVID-19 survivors has been investigated with various results; however, there is limited data documenting outcomes longer than 6 months post-hospitalization. We aimed to investigate the 12-month lasting effects of COVID-19 in hospitalized patients. From October 2020 through March 2021, 92 patients were enrolled. At admission and 1, 3, 6, and 12 months post-hospitalization, demographic, clinical, laboratory and imaging data, and echocardiography and spirometry test results were recorded. Possible cognitive and functional impairment, as well as the quality of life (QoL), were also assessed. In our cohort (median age: 61 years), 31.5% had severe disease at admission, which correlated with worse laboratory findings and a longer hospital stay (p < 0.001). Inflammatory markers were associated with severity initially, but reverted to normal after 3 months. In total, 55%, 37%, 19%, and 15.5% of patients reported at least one persistent symptom in months 1, 3, 6, and 12, respectively, while "brain fog" persisted up to 12 months in 10% of patients. Spirometry and echocardiography tests returned to normal in most patients during the evaluation, and no one had substantial residual disease. Our study provides insight into the long-term effects of COVID-19 on patients' physical and mental health. Despite the lack of significant residual disease or major complications after a year of thorough follow-up, COVID-19 survivors experienced lasting symptoms and a negative impact on their QoL.

Evaluation of repair bond strength of a dental CAD/CAM resin composite after surface treatment with two Er,Cr:YSGG laser protocols following artificial aging.

The aim of the present study was to evaluate the impact of two Er,Cr:YSGG laser surface treatments on the repair bond strength of a dental CAD/CAM resin composite (Brilliant Crios) after artificial aging. Twenty-four resin-based CAD/CAM blocks were cut and 48 rectangular slabs (3 x 12 x 14 mm) were prepared. Preliminary SEM observations indicated the most favorable laser settings regarding surface modification of the tested restorative material. The CAD/CAM specimens were then divided into 4 groups (n=12) based on their surface pretreatment: no treatment, air abraded with 50-µm Al2O3 particles, and laser irradiated with two different protocols using Er,Cr:YSGG laser with average power 3.5 and 4.5 W, pulse repetition rate 35 and 50 Hz, and pulse energy 100 and 90 mJ, respectively. After surface treatments each group followed a bonding protocol with silane and a flowable resin composite was used to prepare 48 microrods. Half of the microrods of each group were subjected on shear bond strength (SBS) test (chisel-shaped blade, load cell of 500 N, crosshead speed of 1 mm/min) after 24 h, while the other half underwent artificial aging (15,000 cycles, 5-55 °C) and then SBS test. The debonded specimens were examined under an optical microscope to determine the failure mode. All specimens were also evaluated using SEM to assess the surface topography after the treatments. The results showed that SBS significantly decreased after thermocycling in all the experimental groups (p<0.05). Control group presented much lower SBS values than the other groups after both 24 h and thermocycling (p<0.05). The highest values of SBS exhibited air-abrasion group (p<0.05), followed by the two laser groups, which did not differ to each other (p>0.05). The results of the current study indicated that Er,Cr:YSGG laser irradiation can be an alternative treatment for repairing the tested resin-based CAD/CAM restorative material.

Retrospective analysis of persistent HyperCKemia with or without muscle weakness in a case series from Greece highlights vast DMD variant heterogeneity.

BACKGROUND: Persistent hyperCKemia results from muscle dysfunction often attributed to genetic alterations of muscle-related genes, such as the dystrophin gene (DMD). Retrospective assessment of findings from DMD analysis, in association with persistent HyperCKemia, was conducted. PATIENTS AND METHODS: Evaluation of medical records from 1354 unrelated cases referred during the period 1996-2021. Assessment of data concerning the detection of DMD gene rearrangements and nucleotide variants. RESULTS: A total of 730 individuals (657 cases, 569 of Greek and 88 of Albanian origins) were identified, allowing an overall estimation of dystrophinopathy incidence at ~1:3800 live male births. The heterogeneous spectrum of 275 distinct DMD alterations comprised exon(s) deletions/duplications, nucleotide variants, and rare events, such as chromosome translocation {t(X;20)}, contiguous gene deletions, and a fused gene involving the DMD and the DOCK8 genes. Ethnic-specific findings include a common founder variant in exon 36 ('Hellenic' variant). CONCLUSIONS: Some 50% of hyperCKemia cases were characterized as dystrophinopathies, highlighting that DMD variants may be considered the most common cause of hyperCKemia in Greece. Delineation of the broad genetic and clinical heterogeneity is fundamental for actionable public health decisions and theragnosis, as well as the establishment of guidelines addressing ethical considerations, especially related to the mild asymptomatic patient subgroup.

Analysis of muscle magnetic resonance imaging of a large cohort of patient with VCP-mediated disease reveals characteristic features useful for diagnosis.

BACKGROUND: The diagnosis of patients with mutations in the VCP gene can be complicated due to their broad phenotypic spectrum including myopathy, motor neuron disease and peripheral neuropathy. Muscle MRI guides the diagnosis in neuromuscular diseases (NMDs); however, comprehensive muscle MRI features for VCP patients have not been reported so far. METHODS: We collected muscle MRIs of 80 of the 255 patients who participated in the "VCP International Study" and reviewed the T1-weighted (T1w) and short tau inversion recovery (STIR) sequences. We identified a series of potential diagnostic MRI based characteristics useful for the diagnosis of VCP disease and validated them in 1089 MRIs from patients with other genetically confirmed NMDs. RESULTS: Fat replacement of at least one muscle was identified in all symptomatic patients. The most common finding was the existence of patchy areas of fat replacement. Although there was a wide variability of muscles affected, we observed a common pattern characterized by the involvement of periscapular, paraspinal, gluteal and quadriceps muscles. STIR signal was enhanced in 67% of the patients, either in the muscle itself or in the surrounding fascia. We identified 10 diagnostic characteristics based on the pattern identified that allowed us to distinguish VCP disease from other neuromuscular diseases with high accuracy. CONCLUSIONS: Patients with mutations in the VCP gene had common features on muscle MRI that are helpful for diagnosis purposes, including the presence of patchy fat replacement and a prominent involvement of the periscapular, paraspinal, abdominal and thigh muscles.

Deep Characterization of a Greek Patient with Desmin-Related Myofibrillar Myopathy and Cardiomyopathy.

Desmin is a class III intermediate filament protein highly expressed in cardiac, smooth and striated muscle. Autosomal dominant or recessive mutations in the desmin gene (DES) result in a variety of diseases, including cardiomyopathies and myofibrillar myopathy, collectively called desminopathies. Here we describe the clinical, histological and radiological features of a Greek patient with a myofibrillar myopathy and cardiomyopathy linked to the c.734A>G,p.(Glu245Gly) heterozygous variant in the DES gene. Moreover, through ribonucleic acid sequencing analysis in skeletal muscle we show that this variant provokes a defect in exon 3 splicing and thus should be considered clearly pathogenic.

Effect of Whitening Toothpastes with Different Active Agents on the Abrasive Wear of Dentin Following Tooth Brushing Simulation.

The aim of this research was to evaluate the abrasive dentin wear that can be induced by three commercial whitening toothpastes following a tooth-brushing simulation (TBS) corresponding to a three-month period. Sixty human canines were selected, and the roots were separated from the crowns. Then the roots were randomly divided into six groups (n = 10) and were submitted to TBS using the following slurries: Group 1-deionized water (RDA = 5); Group 2-ISO dentifrice slurry (RDA = 100); Group 3-a regular toothpaste (RDA = 70); Group 4-a charcoal-containing whitening toothpaste; Group 5-a whitening toothpaste containing blue covasorb and hydrated silica; and Group 6-a whitening toothpaste containing microsilica. Following TBS, surface loss and surface roughness changes were evaluated using confocal microscopy. Additionally, surface morphology and mineral content changes were observed using scanning electron microscopy and energy-dispersive X-ray spectroscopy. The deionized water group presented the lowest surface loss (p < 0.05), while the charcoal-containing toothpaste presented the highest surface loss, followed by ISO dentifrice slurry (p < 0.001). Blue-covasorb-containing and regular toothpastes did not present statistically significant differences (p = 0.245), and neither didmicrosilica-containing toothpaste or ISO dentifrice slurry (p = 0.112). The surface height parameters and surface morphology changes of the experimental groups followed the surface loss patterns, while no differences were detected in mineral content after TBS.Although the charcoal-containing toothpaste exhibited the highest abrasive wear to dentin, according to ISO 11609, all the tested toothpastes exhibited appropriate abrasive behavior towards dentin.

Effect of tooth brushing simulation on the surface properties of various resin-matrix computer-aided design/computer-aided manufacturing ceramics.

PURPOSE: The purpose was to investigate the alterations in surface properties of different resin-matrix CAD/CAM ceramics following tooth brushing simulation (TBS) and compare them with a direct resin composite and a glass ceramic CAD/CAM material. MATERIALS AND METHODS: Four resin-based CAD/CAM restoratives (Brilliant Crios-BR, Lava™ Ultimate-LV, Grandio Blocs-GR and Shofu Block HC-SH), a leucite-reinforced glass ceramic (IPS Empress® CAD-EC) and a resin composite (Filtek™ Z250-FZ) for direct restorations were tested. In particular, surface loss, hardness, roughness and morphology were investigated utilizing confocal microscopy, scanning electron microscopy and nanoindentation tester. TBS was conducted for 4 × 15 min on the surface of the samples and then the changes in their surface properties were evaluated. RESULTS: After TBS, all the experimental groups exhibited surface loss to different extent. FZ and BR presented the highest surface loss, while EC and GR the lowest (p < 0.05). Regarding surface roughness, all the tested materials exhibited increase after TBS (p < 0.05), except LV (p = 0.099). EC presented the lowest Sa values, while FZ and BR the highest (p < 0.05). Changes in surface morphology were in compliance with the results of surface roughness and also surface hardness was correlated with surface loss. CONCLUSIONS: The tested resin-matrix CAD/CAM ceramic restorative materials showed a competent behavior against abrasive forces applied during TBS. Surface loss and roughness changes were material dependent and superior compared to a resin composite for direct restorations, while in comparison with a leucite-reinforced glass ceramic exhibited inferior properties. CLINICAL SIGNIFICANCE: Tooth brushing affected differently the surface of the tested restorative materials. However, the abrasive wear that was induced was negligible. Clinical studies are necessary to ascertain if there is clinical significance of these surface alterations that may demand repair of such restorations.

Skeletal muscle fiber composition may modify the effect of nutrition on body composition in young females.

BACKGROUND AND AIM: The aim of this study was to investigate the hypothesis that healthy, normal-weight females with greater proportions and sizes of the oxidative muscle fibers would also be characterized by a healthier body composition compared with individuals with increased glycolytic fibers, even if both follow similar nutritional plans. METHODS AND RESULTS: Vastus lateralis muscle fiber-type composition, body composition through dual-energy X-ray absorptiometry, and dietary intakes through questionnaire were evaluated in twenty-two young, healthy, non-obese females (age: 21.3±1.8yrs, body mass: 67.5±6.2 kg, body height: 1.66±0.05m, body mass index (BMI): 24.2±2.6  kg m-2). The participants were allocated into two groups according to their type I muscle fibers percentage [high (HI) and low (LI)]. The participants of the LI group were characterized by significantly higher body mass, fat mass, BMI, and cross-sectional and percentage cross-sectional area (%CSA) of type IIx muscle fibers compared with participants of the HI group (p < 0.021). In contrast, the HI group was characterized by higher cross-sectional and %CSA of type I muscle fibers compared with the LI group (p < 0.038). Significant correlations were observed between body fat mass, lean body mass, total energy intake, fat energy intake, and %CSAs of type I and IIx muscle fibers (r: -0.505 to 0.685; p < 0.05). CONCLUSION: In conclusion, this study suggests that muscle fiber composition is an important factor that at least partly could explain the observed differential inter-individual responses of the body composition to nutrition in female individuals. Increased %CSAs of type I muscle fibers seem to act as a protective mechanism against obesity and favor a healthier body composition, neutralizing the negative effect of increased caloric fats intake on body composition, probably because of their greater oxidative metabolic properties and fat utilization capacities. In contrast, female individuals with low type I and high type IIx %CSAs of type I seem to be more metabolically inflexible and dietinduced obesity prone, even if they consume fewer total daily calories and fats.

Skeletal muscle and erythrocyte redox status is associated with dietary cysteine intake and physical fitness in healthy young physically active men.

PURPOSE: To investigate the association between redox status in erythrocytes and skeletal muscle with dietary nutrient intake and markers of physical fitness and habitual physical activity (PA). METHODS: Forty-five young physically active men were assessed for body composition, dietary nutrient intake, muscle strength, cardiorespiratory capacity and habitual PA. Blood and muscle samples were collected to estimate selected redox biomarkers. Partial correlation analysis was used to evaluate the independent relationship of each factor with redox biomarkers. RESULTS: Dietary cysteine intake was positively correlated (p < 0.001) with both erythrocyte (r = 0.697) and muscle GSH (0.654, p < 0.001), erythrocyte reduced/oxidized glutathione ratio (GSH/GSSG) (r = 0.530, p = 0.001) and glutathione reductase (GR) activity (r = 0.352, p = 0.030) and inversely correlated with erythrocyte protein carbonyls (PC) levels (r = - 0.325; p = 0.046). Knee extensors eccentric peak torque was positively correlated with GR activity (r = 0.355; p = 0.031) while, one-repetition maximum in back squat exercise was positively correlated with erythrocyte GSH/GSSG ratio (r = 0.401; p = 0.014) and inversely correlated with erythrocyte GSSG and PC (r = - 0.441, p = 0.006; r = - 0.413, p = 0.011 respectively). Glutathione peroxidase (GPx) activity was positively correlated with step count (r = 0.520; p < 0.001), light (r = 0.406; p = 0.008), moderate (r = 0.417; p = 0.006), moderate-to-vigorous (r = 0.475; p = 0.001), vigorous (r = 0.352; p = 0.022) and very vigorous (r = 0.326; p = 0.035) PA. Muscle GSSG inversely correlated with light PA (r = - 0.353; p = 0.022). CONCLUSION: These results indicate that dietary cysteine intake may be a critical element for the regulation of glutathione metabolism and redox status in two different tissues pinpointing the independent significance of cysteine for optimal redox regulation. Musculoskeletal fitness and PA levels may be predictors of skeletal muscle, but not erythrocyte, antioxidant capacity. TRIAL REGISTRATION: Registry: ClinicalTrials.gov, identifier: NCT03711838, date of registration: October 19, 2018.