Elevated liver glycogenolysis mediates higher blood glucose during acute exercise in Barth syndrome.

Unlabelled: Barth syndrome (BTHS) is an X-linked recessive genetic disorder due to mutations in the Tafazzin (TAFAZZIN) gene that lead to cardiac and skeletal muscle mitochondrial dysfunction. Previous studies in humans with BTHS demonstrate that the defects in muscle mitochondrial oxidative metabolism result in an enhanced reliance on anaerobic metabolism during exercise to meet energy demands of muscular work. During exercise, the liver normally increases glucose production via glycogenolysis and gluconeogenesis to match the elevated rate of muscle glucose uptake and meet the ATP requirements of working muscle.

Short-term Intensive Lifestyle Therapy in a Worksite Setting Improves Cardiometabolic Health in People With Obesity.

Context: The Pritikin Program, which provides intensive lifestyle therapy, has been shown to improve cardiometabolic outcomes when provided as a residential program.

Objective: The purpose of the present study was to conduct a short-term, randomized, controlled trial to evaluate the feasibility and clinical efficacy of treatment with the Pritikin Program in an outpatient worksite setting.

Methods: Cardiometabolic outcomes were evaluated in people with overweight/obesity and ≥2 metabolic abnormalities (high triglycerides, low high-density lipoprotein (HDL) cholesterol, high blood pressure, HbA1c > 5.

The Effect of Smoking Status on Perioperative Morbidity and Mortality after Open and Endovascular Abdominal Aortic Aneurysm Repair.

Background: This study quantifies the extent to which active tobacco smoking is deleterious toward outcomes following open and endovascular abdominal aortic aneurysm (AAA) repair.

Methods: Open and endovascular AAA repairs between January 2003 and June 2020 in the Vascular Quality Initiative were queried. Rupture, symptomatic status, and lack of 90 day follow-up were exclusions.

Do lifestyle factors and quality of life differ in people with metabolically healthy and unhealthy obesity?

Background And Objectives: Although obesity is typically associated with metabolic co-morbidities, some people with obesity do not develop metabolic abnormalities. We evaluated whether modifiable lifestyle factors (i.e.

Protocol for Transfusion of Packed Red Blood Cells in the Brazilian Air Medical Service.

Objective: Helicopter emergency medical services are part of many emergency health care systems, ensuring specialized treatment on site. The objective of this study was to develop and validate a protocol for the use of packed red blood cells in advanced support helicopter air medical care in Brazil.

Methods: This was a methodological validation study with a quantitative approach developed in 2 stages (ie, protocol development and content validity using the Delphi technique) by 24 judges.

Effect of -3 long-chain polyunsaturated fatty acid intake on the eicosanoid profile in individuals with obesity and overweight: a systematic review and meta-analysis of clinical trials.

Dietary -3 polyunsaturated fatty acids (PUFAs) present beneficial effects on counteracting inflammation status, displaying a critical anti-inflammatory role and maintaining physiological homeostasis in obesity. The primary objective of this systematic review was to evaluate the effect of -3 PUFAs intake on the eicosanoid profile of people with obesity and overweight. The search strategy on Embase, Scopus, PubMed, Web of Science, Cochrane Library, Google Scholar and ProQuest was undertaken until November 2019 and updated January 2021.

Myocardial Lipin 1 knockout in mice approximates cardiac effects of human LPIN1 mutations.

Lipin 1 is a bifunctional protein that is a transcriptional regulator and has phosphatidic acid (PA) phosphohydrolase activity, which dephosphorylates PA to generate diacylglycerol. Human lipin 1 mutations lead to episodic rhabdomyolysis, and some affected patients exhibit cardiac abnormalities, including exercise-induced cardiac dysfunction and cardiac triglyceride accumulation. Furthermore, lipin 1 expression is deactivated in failing heart, but the effects of lipin 1 deactivation in myocardium are incompletely understood.

Increased Adipose Tissue Fibrogenesis, Not Impaired Expandability, Is Associated With Nonalcoholic Fatty Liver Disease.

Background And Aims: It is proposed that impaired expansion of subcutaneous adipose tissue (SAT) and an increase in adipose tissue (AT) fibrosis causes ectopic lipid accumulation, insulin resistance (IR), and metabolically unhealthy obesity. We therefore evaluated whether a decrease in SAT expandability, assessed by measuring SAT lipogenesis (triglyceride [TG] production), and an increase in SAT fibrogenesis (collagen production) are associated with NAFLD and IR in persons with obesity.

Approach And Results: In vivo abdominal SAT lipogenesis and fibrogenesis, expression of SAT genes involved in extracellular matrix (ECM) formation, and insulin sensitivity were assessed in three groups of participants stratified by adiposity and intrahepatic TG (IHTG) content: (1) healthy lean with normal IHTG content (Lean-NL; n = 12); (2) obese with normal IHTG content and normal glucose tolerance (Ob-NL; n = 25); and (3) obese with NAFLD and abnormal glucose metabolism (Ob-NAFLD; n = 25).

Short Term High-Repetition Back Squat Protocol Does Not Improve 5-km Run Performance.

The purpose of this study was to evaluate the hypothesis that a novel high-repetition, low-resistance back squat training protocol, designed to stimulate high-intensity interval training, improves 5-km run performance. Fifteen runners [4 male, 11 female; 150 + minutes of endurance exercise/week; age = 22.7 ± 2.

Synthesis and Stability of Actinium-225 Endohedral Fullerenes, Ac@C.

We report the first synthesis of Ac ( = 10 days) endohedral fullerenes,Ac@C. The Ac@C was produced with a 12 ± 2% efficiency by applying an electrical arc discharge between a source of α-particle emitter Ac (∼1 mCi, electroplated on a Pt disk) and a thin coat of "preformed" C on an Al disk (C thickness of ∼0.25 mg/cm).

Implementation of the protocol of nursing care in trauma in aeromedical service.

Objective: To analyze the implementation of a nursing care protocol for trauma patients before, during and after the flight.

Method: A cross-sectional quantitative study carried out in an aeromedical service, using a checklist with 106 care: 79 before flight, 25 during, and 2 after flight. 97 patients were included in the study.

Insulin resistance drives hepatic de novo lipogenesis in nonalcoholic fatty liver disease.

BACKGROUNDAn increase in intrahepatic triglyceride (IHTG) is the hallmark feature of nonalcoholic fatty liver disease (NAFLD) and is decreased by weight loss. Hepatic de novo lipogenesis (DNL) contributes to steatosis in individuals with NAFLD. The physiological factors that stimulate hepatic DNL and the effect of weight loss on hepatic DNL are not clear.

Structural Characteristics, Population Analysis, and Binding Energies of [An(NO)] (with An = Ac to Lr).

Efficient predictive capabilities are essential for the actinide series since regulatory constraints for radioactive work, associated costs needed for specialized facilities, and the short half-lives of many actinides present great challenges in laboratory settings. Improved predictive accuracy is advantageous for numerous applications including the optimization and design of separation agents for nuclear fuel and waste. One limitation of calculations in support of these applications is that the large variations observed from predictions obtained with currently available methods can make comparisons across studies uncertain.

Structural Analysis of the Complexation of Uranyl, Neptunyl, Plutonyl, and Americyl with Cyclic Imide Dioximes.

Knowledge-based design of extracting agents for selective binding of actinides is essential in stock-pile stewardship, environmental remediation, separations, and nuclear fuel disposal. Robust computational protocols are critical for in depth understanding of structural properties and to further advance the design of selective ligands. In particular, rapid radiochemical separations require predictive capabilities for binding in the gas phase.

A mutation in Site-1 Protease is associated with a complex phenotype that includes episodic hyperCKemia and focal myoedema.

Background: Site-1 Protease (S1P) is a Golgi-resident protein required for the activation of regulatory proteins that drive key cellular functions, including, the unfolded protein response (UPR) and lipid and cholesterol biosynthesis. While disruptions in S1P function have been widely characterized in animal models, to date, the implications of disrupted S1P function in human disease states are not completely known.

Methods: The patient and both parents underwent whole exome and mitochondrial DNA sequencing, and Sanger sequencing was used to confirm the mutation.

Blunted fat oxidation upon submaximal exercise is partially compensated by enhanced glucose metabolism in children, adolescents, and young adults with Barth syndrome.

Barth syndrome (BTHS) is a rare X-linked condition resulting in abnormal mitochondria, cardioskeletal myopathy, and growth delay; however, the effects of BTHS on substrate metabolism regulation and their relationships with tissue function in humans are unknown. We sought to characterize glucose and fat metabolism during rest, submaximal exercise, and postexercise rest in children, adolescents, and young adults with BTHS and unaffected controls and examine their relationships with cardioskeletal energetics and function. Children/adolescents and young adults with BTHS (n = 29) and children/adolescent and young adult control participants (n = 28, total n = 57) underwent an infusion of 6'6'H2 glucose and U- C palmitate and indirect calorimetry during rest, 30-minutes of moderate exercise (50% ), and recovery.

Hepatic monoacylglycerol acyltransferase 1 is induced by prolonged food deprivation to modulate the hepatic fasting response.

During prolonged fasting, the liver plays a central role in maintaining systemic energy homeostasis by producing glucose and ketones in processes fueled by oxidation of fatty acids liberated from adipose tissue. In mice, this is accompanied by transient hepatic accumulation of glycerolipids. We found that the hepatic expression of monoacylglycerol acyltransferase 1 (), an enzyme with monoacylglycerol acyltransferase (MGAT) activity that produces diacyl-glycerol from monoacylglycerol, was significantly increased in the liver of fasted mice compared with mice given ad libitum access to food.

Parametric study on the production of the GAGG:Ce and LSO:Ce multicomponent oxide scintillator materials through use of a planetary ball mill.

The material presented herein focuses on the exploration of the production of gadolinium aluminum gallium garnet and lutetium oxyorthosilicate doped with cerium (GAGG:Ce and LSO:Ce, respectively) through mechanochemical means. Multiple parameters are explored including mass of starting material, ball size, rotational speed of the mill, number of balls employed, and material used for the milling container. Theoretical calculations were conducted using a pre-established equation and showed that, when all other parameters were held constant, in scenarios where (1) a smaller mass of sample, (2) faster revolutions per minute, (3) greater numbers of balls, or (4) a greater density of the material used for the vials and balls were employed, there should be higher energies imparted to the system.

Loss of lipin 1-mediated phosphatidic acid phosphohydrolase activity in muscle leads to skeletal myopathy in mice.

Lipin 1 regulates glycerolipid homeostasis by acting as a phosphatidic acid phosphohydrolase (PAP) enzyme in the triglyceride-synthesis pathway and by regulating transcription factor activity. Mutations in human lipin 1 are a common cause of recurrent rhabdomyolysis in children. Mice with constitutive whole-body lipin 1 deficiency have been used to examine mechanisms connecting lipin 1 deficiency to myocyte injury.

The impact of diet-induced hepatic steatosis in a murine model of hepatic ischemia/reperfusion injury.

The prevalence of obesity-associated nonalcoholic fatty liver disease has significantly increased over the past decade, and end-stage liver disease secondary to nonalcoholic steatohepatitis has become 1 of the most common indications for liver transplantation. This both increases the demand for organs and decreases the availability of donor livers deemed suitable for transplantation. Although in the past many steatotic livers were discarded due to concerns over enhanced susceptibility to ischemia/reperfusion injury (IRI) and organ failure, the discrepancy between supply and demand has resulted in increasing use of expanded criteria donor organs including steatotic livers.