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https://www.readbyqxmd.com/read/29786180/-bartter-syndrome-severe-rare-orphan-kidney-disease-a-step-towards-therapy-through-pharmacogenetic-and-epidemiological-studies
#1
EDITORIAL
Elena Conte, Paola Imbrici, Dalila Sahbani, Antonella Liantonio, Diana Conte
Bartter syndromes (BS) types 1-5 are rare salt-losing tubulopathies presenting with overlapping clinical phenotypes including marked salt wasting and hypokalemia leading to polyuria, polydipsia, volume contraction, muscle weakness and growth retardation. These diseases are due to an impairment of sodium, potassium, chloride reabsorption caused by mutations in genes encoding for ion channel or transporters expressed in specific nephron tubule segments. Particularly, BS type 3 is a clinically heterogeneous form caused by mutations in CLCNKB gene which encodes the ClC-Kb chloride channel involved in NaCl reabsorption in the renal tubule...
May 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/29785570/phase-1b-investigation-of-the-mek-inhibitor-binimetinib-in-patients-with-advanced-or-metastatic-biliary-tract-cancer
#2
R S Finn, D H Ahn, M M Javle, B R Tan, C D Weekes, J C Bendell, A Patnaik, G N Khan, D Laheru, R Chavira, J Christy-Bittel, E Barrett, M B Sawyer, Tanios S Bekaii-Saab
Background The MAPK pathway plays a central role in regulation of several cellular processes, and its dysregulation is a hallmark of biliary tract cancer (BTC). Binimetinib (MEK162), a potent, selective oral MEK1/2 inhibitor, was assessed in patients with advanced BTC. Patients and Methods An expansion cohort study in patients who received ≤1 line of therapy for advanced BTC was conducted after determination of the maximum tolerated dose in this Phase 1 trial. Patients received binimetinib 60 mg twice daily...
May 22, 2018: Investigational New Drugs
https://www.readbyqxmd.com/read/29776315/aerobic-training-reduces-oxidative-stress-in-skeletal-muscle-of-rats-exposed-to-air-pollution-and-supplemented-with-chromium-picolinate
#3
Bruna Marmett, Ramiro Barcos Nunes, Kellen Sábio de Souza, Pedro Dal Lago, Cláudia Ramos Rhoden
OBJECTIVE: The purpose of this study was to investigate the effects of chromium picolinate (CrPic) supplementation associated with aerobic exercise using measures of oxidative stress in rats exposed to air pollution. METHODS: Sixty-one male Wistar rats were divided into eight groups: residual oil fly ash (ROFA) exposure and sedentary (ROFA-SED); ROFA exposure, sedentary and supplemented (ROFA-SED-CrPic); ROFA exposure and trained (ROFA-AT); ROFA exposure, supplemented and trained (ROFA-AT-CrPic); sedentary (Sal-SED); sedentary and supplemented (Sal-SED-CrPic); trained (Sal-AT); and supplemented and trained (Sal-AT-CrPic)...
December 2018: Redox Report: Communications in Free Radical Research
https://www.readbyqxmd.com/read/29773650/the-exercise-inducible-bile-acid-receptor-tgr5-improves-skeletal-muscle-function-in-mice
#4
Takashi Sasaki, Ayane Kuboyama, Moeko Mita, Shotaro Murata, Makoto Shimizu, Jun Inoue, Kazutoshi Mori, Ryuichiro Sato
TGR5 (also known as G protein-coupled bile acid receptor 1, GPBAR1) is a G protein-coupled bile acid receptor that is expressed in many diverse tissues. TGR5 is involved in various metabolic processes, including glucose metabolism and energy expenditure; however, TGR5's function in skeletal muscle is not fully understood. Using both gain- and loss-of-function mouse models, we demonstrate here that Tgr5 activation promotes muscle cell differentiation and muscle hypertrophy. Both young and old transgenic mice with muscle-specific Tgr5 expression exhibited increased muscle strength...
May 17, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29772836/soybean-hop-alleviates-estrogen-deficiency-related-bone-loss-and-metabolic-dysfunction-in-ovariectomized-rats-fed-a-high-fat-diet
#5
Dasom Noh, Yeni Lim, Hansol Lee, Hyejin Kim, Oran Kwon
Soybeans and hops have been traditionally used as a natural estrogen replacement therapy and their major active ingredients, isoflavones and prenylflavanones, are known to have estrogenic/antiestrogenic effects depending on the target organ. However, their potential benefits are still subject to controversies. The present study investigated the dual effect of soy isoflavones plus hop prenylflavanones (Soy-Hop) on bone loss and metabolic dysfunction under estrogen deficient condition. Rats were sham-operated ( n = 10) or ovariectomized (OVX; n = 40) and then fed a high-fat diet (HFD) to develop hyperlipidemia in OVX rats within the experimental period of 8 weeks...
May 17, 2018: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/29767564/interorgan-metabolic-crosstalk-in-human-insulin-resistance
#6
Sofiya Gancheva, Tomas Jelenik, Elisa Álvarez-Hernández, Michael Roden
Excessive energy intake and reduced energy expenditure drive the development of insulin resistance and metabolic diseases such as obesity and type 2 diabetes mellitus. Metabolic signals derived from dietary intake or secreted from adipose tissue, gut, and liver contribute to energy homeostasis. Recent metabolomic studies identified novel metabolites and enlarged our knowledge on classic metabolites. This review summarizes the evidence of their roles as mediators of interorgan crosstalk and regulators of insulin sensitivity and energy metabolism...
July 1, 2018: Physiological Reviews
https://www.readbyqxmd.com/read/29767150/effect-of-storage-time-on-the-characteristics-of-corn-and-efficiency-of-its-utilization-in-broiler-chickens
#7
Dafei Yin, Jianmin Yuan, Yuming Guo, Lee I Chiba
Corn is one of the staple food and feed ingredients in China, therefore its storage is of particular importance. Corn is typically stored for 2 or more years in national barns before it is sold as a food or feed ingredient. However, the effects of stored corn in national barns on the animal performance and nutrient utilization have not been investigated thus far. This study attempted to determine the effects of storage time on the chemical and physical characteristics of corn and its nutritional value, broiler growth performance, and meat quality...
September 2017: Animal nutrition
https://www.readbyqxmd.com/read/29767088/interactive-effect-of-dietary-vitamin-e-and-inorganic-mercury-on-growth-performance-and-bioaccumulation-of-mercury-in-juvenile-olive-flounder-paralichthys-olivaceus-treated-with-mercuric-chloride
#8
Mohammad Moniruzzaman, Jun-Ho Lee, Jin-Hyeok Lee, Seonghun Won, Jim H Damusaru, Sungchul C Bai
A 6-week feeding trial was carried out to evaluate the effects of dietary vitamin E (dl-α-tocopheryl acetate, TA) on growth and mercury (Hg) accumulation in juvenile olive flounder ( Paralichthys olivaceus ) treated with mercuric chloride (HgCl2 ). Vitamin E and HgCl2 were added to the semi-purified basal diet. Six semi-purified diets in a 2 × 3 factorial design were formulated to contain 2 levels of Hg (0 or 20 mg HgCl2 /kg diet) and 3 levels of vitamin E (0, 100, or 200 mg TA/kg diet). Experimental fish ( n  = 360, 9...
September 2017: Animal nutrition
https://www.readbyqxmd.com/read/29762881/colchicine-a-microtubule-disassembling-drug-in-the-therapy-of-cardiovascular-diseases
#9
George N Chaldakov
Colchicum autumnale, from which colchicine has been isolated more than 100 years ago, has been used as a treatment for pain and swelling for thousands of years. It is one of the few drugs known from that time period whose use has survived to modernity. Over the past decades, advances in the knowledge of (i) cytoskeletal microtubules (МТ), and (ii) anti-inflammatory and anti-fibrotic effects of colchicine, a classical MT-disassembling (tubulin-targeting) agent, have led to potential new uses for this very old drug extended beyond acute gouty arthritis and familial Mediterranean fever...
May 15, 2018: Cell Biology International
https://www.readbyqxmd.com/read/29762790/dietary-supplementation-with-clostridium-butyricum-modulates-serum-lipid-metabolism-meat-quality-and-the-amino-acid-and-fatty-acid-composition-of-peking-ducks
#10
Yanhan Liu, Yiyu Li, Xiancheng Feng, Zhong Wang, Zhaofei Xia
The aim of this study was to investigate the effects of Clostridium butyricum (C. butyricum) on the performance, serum lipid metabolism, muscle morphology, meat quality, and fatty acid profiles of Peking ducks. A total of 1,500 Peking ducks were randomly divided into five groups with five replicates and were fed a non-antibiotic basal diet (Control) or a basal diet supplemented with either 200, 400, or 600 mg/kg of C. butyricum (2.0 × 109 CFU/g) or 150 mg of aureomycin/kg for 42 d. Compared with the control group, supplementation with C...
May 14, 2018: Poultry Science
https://www.readbyqxmd.com/read/29761054/is-high-intensity-interval-cycling-feasible-and-more-beneficial-than-continuous-cycling-for-knee-osteoarthritic-patients-results-of-a-randomised-control-feasibility-trial
#11
Justin W Keogh, Josephine Grigg, Christopher J Vertullo
Background: Knee osteoarthritis (OA) patients often suffer joint pain and stiffness, which contributes to negative changes in body composition, strength, physical performance (function), physical activity and health-related quality of life. To reduce these symptoms and side effects of knee OA, moderate-intensity continuous training (MICT) cycling is often recommended. While resistance training is considered the optimal form of training to improve sarcopenic outcomes, it imposes higher joint loads and requires supervision, either initially or continuously by trained exercise professionals...
2018: PeerJ
https://www.readbyqxmd.com/read/29760645/systems-analysis-of-human-visuo-myoelectric-control-facilitated-by-anodal-transcranial-direct-current-stimulation-in-healthy-humans
#12
Vinh Kha, Aguida S Foerster, Susan Bennett, Michael A Nitsche, Filip Stefanovic, Anirban Dutta
Induction of neuroplasticity by transcranial direct current stimulation (tDCS) applied to the primary motor cortex facilitates motor learning of the upper extremities in healthy humans. The impact of tDCS on lower limb functions has not been studied extensively so far. In this study, we applied a system identification approach to investigate the impact of anodal transcranial direct current stimulation of the leg area of the motor cortex via the human visuo-myoelectric controller. The visuo-myoelectric reaching task (VMT) involves ballistic muscle contraction after a visual cue...
2018: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/29758954/the-use-of-transcranial-magnetic-stimulation-to-evaluate-cortical-excitability-of-lower-limb-musculature-challenges-and-opportunities
#13
Trisha M Kesar, James W Stinear, Steven L Wolf
Neuroplasticity is a fundamental yet relatively unexplored process that can impact rehabilitation of lower extremity (LE) movements. Transcranial magnetic stimulation (TMS) has gained widespread application as a non-invasive brain stimulation technique for evaluating neuroplasticity of the corticospinal pathway. However, a majority of TMS studies have been performed on hand muscles, with a paucity of TMS investigations focused on LE muscles. This perspective review paper proposes that there are unique methodological challenges associated with using TMS to evaluate corticospinal excitability of lower limb muscles...
May 5, 2018: Restorative Neurology and Neuroscience
https://www.readbyqxmd.com/read/29754437/reduced-fiber-size-capillary-supply-and-mitochondrial-activity-in-constitutional-thinness-skeletal-muscle
#14
Bogdan Galusca, Julien Verney, Emmanuelle Meugnier, Yiin Ling, Pascal Edouard, Leonard Feasson, Marion Ravelojaona, Hubert Vidal, Bruno Estour, Natacha Germain
AIM: Constitutional thinness (CT) is a rare condition of natural low bodyweight, with no psychological issues, no marker of undernutrition and a resistance to weight gain. This study evaluated the skeletal muscle phenotype of CT women by comparison to a normal BMI control group. METHODS: 10 CT women (BMI< 17.5 kg/m2) and 10 female controls (BMI: 18.5-25 kg/m2) underwent metabolic and hormonal assessment along with muscle biopsies to analyse the skeletal muscular fibers pattern, capillarity, enzymes activities and transcriptomics...
May 13, 2018: Acta Physiologica
https://www.readbyqxmd.com/read/29751322/gait-deviations-in-duchenne-muscular-dystrophy-part-2-statistical-non-parametric-mapping-to-analyze-gait-deviations-in-children-with-duchenne-muscular-dystrophy
#15
Marije Goudriaan, Marleen Van den Hauwe, Cristina Simon-Martinez, Catherine Huenaerts, Guy Molenaers, Nathalie Goemans, Kaat Desloovere
BACKGROUND: Prolonged ambulation is considered important in children with Duchenne muscular dystrophy (DMD). However, previous studies analyzing DMD gait were sensitive to false positive outcomes, caused by uncorrected multiple comparisons, regional focus bias, and inter-component covariance bias. Also, while muscle weakness is often suggested to be the main cause for the altered gait pattern in DMD, this was never verified. RESEARCH QUESTION: Our research question was twofold: 1) are we able to confirm the sagittal kinematic and kinetic gait alterations described in a previous review with statistical non-parametric mapping (SnPM)? And 2) are these gait deviations related to lower limb weakness? METHODS: We compared gait kinematics and kinetics of 15 children with DMD and 15 typical developing (TD) children (5-17 years), with a two sample Hotelling's T2 test and post-hoc two-tailed, two-sample t-test...
April 30, 2018: Gait & Posture
https://www.readbyqxmd.com/read/29750730/the-changing-landscape-of-motor-neuron-disease-imaging-the-transition-from-descriptive-studies-to-precision-clinical-tools
#16
Peter Bede, Giorgia Querin, Pierre-François Pradat
PURPOSE OF REVIEW: Neuroimaging in motor neuron disease (MND) has traditionally been seen as an academic tool with limited direct relevance to individualized patient care. This has changed radically in recent years as computational imaging has emerged as a viable clinical tool with true biomarker potential. This transition is not only fuelled by technological advances but also by important conceptual developments. RECENT FINDINGS: The natural history of MND is now evaluated by presymptomatic, postmortem and multi-timepoint longitudinal imaging studies...
May 9, 2018: Current Opinion in Neurology
https://www.readbyqxmd.com/read/29750601/allelic-heterogeneity-of-lamin-a-c-phenotypes-and-molecular-mechanism-behind-it
#17
Jelena Perovanovic, Eric P Hoffman
Mutations in the LMNA gene cause a broad range of clinical syndromes that show tissue-restricted abnormalities of post mitotic tissues, such as muscle, nerve, heart, and adipose tissue. Mutations in other nuclear envelope proteins cause clinically overlapping disorders. The majority of mutations are dominant single amino acid changes (toxic protein produced by the single mutant gene), and patients are heterozygous with both normal and abnormal proteins. Experimental support has been provided for different models of cellular pathogenesis in nuclear envelope diseases, including changes in heterochromatin formation at the nuclear membrane (epigenomics), changes in the timing of steps during terminal differentiation of cells, and structural abnormalities of the nuclear membrane...
May 11, 2018: Physiological Genomics
https://www.readbyqxmd.com/read/29748654/body-composition-and-cardiometabolic-health-the-need-for-novel-concepts
#18
REVIEW
Anja Bosy-Westphal, Wiebke Braun, Corinna Geisler, Kristina Norman, Manfred J Müller
It seems reasonable that overweight and obesity should be defined based on body composition rather than indirect indices like BMI or waist circumference. The use of conventional parameters like fat mass or visceral fat is however of similar and limited value for disease risk prediction at the population level and does not contribute much beyond the use of simple BMI or waist circumference. This conundrum may be partly explained by using complex phenotypes (e.g., Metabolic Syndrome or whole body insulin resistance) rather than more disease-specific outcomes like liver- and muscle insulin resistance...
May 2018: European Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/29747053/use-of-acellular-collagen-matrix-for-the-closure-of-the-open-oral-wound-in-bone-regeneration
#19
Dragan Stankovic, Milica Labudovic-Borovic, Radoslav Radosavljevic, Milos Marinkovic, Esma R Isenovic
Mucograft is collagen matrix was designed for use in open healing situations due to its compact outer layer. The technique presented in this article is an attempt to avail this attribute for covering open oral wound in guided bone regeneration (GBR) procedure. The essential idea of this technique is to avoid scoring of periosteum, submucosa and muscle layer for buccal flap advancement. Therefore, we used mucograft to cover bone substitute and barrier membrane in GBR surgical procedure. Thus, we avoided periostal-releasing incisions (PRI) and gained reposition of the flap to original level without impairing the attached keratinized gingiva...
May 7, 2018: Journal of Stomatology, Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/29744334/selecting-incision-dominant-cases-for-robotic-liver-resection-towards-outpatient-hepatectomy-with-rapid-recovery
#20
Laleh G Melstrom, Susanne G Warner, Yanghee Woo, Virginia Sun, Byrne Lee, Gagandeep Singh, Yuman Fong
Background: The premise of minimally invasive surgery (MIS) is to minimize facial and muscle injury in order to enhance recovery from surgery. Robotic MIS surgery for resection of tumors in solid organs is gaining traction, though clear superiority of this approach is lacking and robotic surgery is more expensive. Our philosophy in robotically-assisted hepatectomy has been to employ this approach for cases where location of tumors make difficult a classical laparoscopic approach (superior/posterior tumors), and cases where the incision for an open operation dominates the course of recovery...
April 2018: Hepatobiliary Surgery and Nutrition
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