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Branch chain amino acids

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https://www.readbyqxmd.com/read/28212548/branched-chain-amino-acids-prevent-hepatic-fibrosis-and-development-of-hepatocellular-carcinoma-in-a-non-alcoholic-steatohepatitis-mouse-model
#1
Kai Takegoshi, Masao Honda, Hikari Okada, Riuta Takabatake, Naoto Matsuzawa-Nagata, Jean S Campbell, Masashi Nishikawa, Tetsuro Shimakami, Takayoshi Shirasaki, Yoshio Sakai, Taro Yamashita, Toshinari Takamura, Takuji Tanaka, Shuichi Kaneko
Oral supplementation with branched-chain amino acids (BCAA; leucine, isoleucine, and valine) in patients with liver cirrhosis potentially suppresses the incidence of hepatocellular carcinoma (HCC) and improves event-free survival. However, the detailed mechanisms of BCAA action have not been fully elucidated. BCAA were administered to atherogenic and high-fat (Ath+HF) diet-induced nonalcoholic steatohepatitis (NASH) model mice. Liver histology, tumor incidence, and gene expression profiles were evaluated. Ath+HF diet mice developed hepatic tumors at a high frequency at 68 weeks...
February 13, 2017: Oncotarget
https://www.readbyqxmd.com/read/28212285/transcriptomic-analysis-reveals-selective-metabolic-adaptation-of-streptococcus-suis-to-porcine-blood-and-cerebrospinal-fluid
#2
Anna Koczula, Michael Jarek, Christian Visscher, Peter Valentin-Weigand, Ralph Goethe, Jörg Willenborg
Streptococcus suis is a zoonotic pathogen that can cause severe pathologies such as septicemia and meningitis in its natural porcine host as well as in humans. Establishment of disease requires not only virulence of the infecting strain but also an appropriate metabolic activity of the pathogen in its host environment. However, it is yet largely unknown how the streptococcal metabolism adapts to the different host niches encountered during infection. Our previous isotopologue profiling studies on S. suis grown in porcine blood and cerebrospinal fluid (CSF) revealed conserved activities of central carbon metabolism in both body fluids...
February 15, 2017: Pathogens
https://www.readbyqxmd.com/read/28209124/rna-seq-and-metabolomic-analyses-of-akt1-mediated-muscle-growth-reveals-regulation-of-regenerative-pathways-and-changes-in-the-muscle-secretome
#3
Chia-Ling Wu, Yoshinori Satomi, Kenneth Walsh
BACKGROUND: Skeletal muscle is a major regulator of systemic metabolism as it serves as the major site for glucose disposal and the main reservoir for amino acids. With aging, cachexia, starvation, and myositis, there is a preferential loss of fast glycolytic muscle fibers. We previously reported a mouse model in which a constitutively-active Akt transgene is induced to express in a subset of muscle groups leading to the hypertrophy of type IIb myofibers with an accompanying increase in strength...
February 16, 2017: BMC Genomics
https://www.readbyqxmd.com/read/28207919/tardive-dyskinesia-21st-century-may-bring-new-treatments-to-a-forgotten-disorder
#4
Gopalkumar Rakesh, Andrew Muzyk, Steven T Szabo, Sanjay Gupta, Chi-Un Pae, Prakash Masand
BACKGROUND: Tardive dyskinesia (TD) is a condition that was first described nearly 60 years ago. Despite the tremendous amount of research on pathophysiology and numerous follow-up studies to understand the course and prognosis of TD, effective treatment of the condition continued to elude us until recently. METHODS: We review and summarize clinical literature on TD, as well as provide an update on new treatments available. RESULTS: The emergence of tetrabenazine as a treatment modality decreased suffering and improved quality of life for patients...
February 1, 2017: Annals of Clinical Psychiatry: Official Journal of the American Academy of Clinical Psychiatrists
https://www.readbyqxmd.com/read/28202214/lethal-neonatal-case-and-review-of-primary-short-chain-enoyl-coa-hydratase-sceh-deficiency-associated-with-secondary-lymphocyte-pyruvate-dehydrogenase-complex-pdc-deficiency
#5
Jirair K Bedoyan, Samuel P Yang, Sacha Ferdinandusse, Rhona M Jack, Alexander Miron, George Grahame, Suzanne D DeBrosse, Charles L Hoppel, Douglas S Kerr, Ronald J A Wanders
Mutations in ECHS1 result in short-chain enoyl-CoA hydratase (SCEH) deficiency which mainly affects the catabolism of various amino acids, particularly valine. We describe a case compound heterozygous for ECHS1 mutations c.836T>C (novel) and c.8C>A identified by whole exome sequencing of proband and parents. SCEH deficiency was confirmed with very low SCEH activity in fibroblasts and nearly absent immunoreactivity of SCEH. The patient had a severe neonatal course with elevated blood and cerebrospinal fluid lactate and pyruvate concentrations, high plasma alanine and slightly low plasma cystine...
February 2, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28199774/sarcopenia-predicts-minimal-hepatic-encephalopathy-in-patients-with-liver-cirrhosis
#6
Tatsunori Hanai, Makoto Shiraki, Satoshi Watanabe, Takahiro Kochi, Kenji Imai, Atsushi Suetsugu, Koji Takai, Hisataka Moriwaki, Masahito Shimizu
AIM: Minimal hepatic encephalopathy (MHE) and sarcopenia impair the health-related quality of life and prognosis in patients with liver cirrhosis; however, the relationship between MHE and sarcopenia remains unclear. The aim of this study was to investigate their relationship and to identify the predictors of MHE in cirrhotic patients. METHODS: This retrospective study evaluated 120 cirrhotic patients who were tested for MHE and sarcopenia. MHE was diagnosed by using the computer-aided neuropsychiatric test...
February 15, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28199757/genetic-enzymatic-and-metabolite-profiling-of-the-lactobacillus-casei-group-reveals-strain-biodiversity-and-potential-applications-for-flavour-diversification
#7
Ewelina Stefanovic, Kieran N Kilcawley, Mary C Rea, Gerald F Fitzgerald, Olivia McAuliffe
AIMS: The Lactobacillus casei group represents a widely explored group of lactic acid bacteria, characterised by a high level of biodiversity. In this study, the genetic and phenotypic diversity of a collection of more than 300 isolates of the L. casei group and their potential to produce volatile metabolites important for flavour development in dairy products was examined. METHODS AND RESULTS: Following confirmation of species by 16S rRNA PCR, the diversity of the isolates was determined by pulsed field gel electrophoresis...
February 15, 2017: Journal of Applied Microbiology
https://www.readbyqxmd.com/read/28197878/two-homozygous-mutations-in-the-exon-5-of-bckdhb-gene-that-may-cause-the-classic-form-of-maple-syrup-urine-disease
#8
Ling Su, Zhikun Lu, Fatao Li, Yongxian Shao, Huiying Sheng, Yanna Cai, Li Liu
Maple syrup urine disease (MSUD) is a rare autosomal recessive genetic disorder caused by defects in the catabolism of the branched-chain amino acids (BCAAs). Classic form of MSUD (CMSUD) is caused by mutations in BCKDHA, BCKDHB, DBT genes mostly. In this study, we analyzed the clinical and genetic characteristics of two patients with CMSUD. Two homozygous mutations, c.517G > T (p.Asp173Tyr) and c.503G > A (p.Arg168His), both in the exon 5 of BCKDHB were detected respectively. The novel mutation p...
February 15, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28194962/high-speed-quantitative-uplc-ms-analysis-of-multiple-amines-in-human-plasma-and-serum-via-precolumn-derivatization-with-6-aminoquinolyl-n-hydroxysuccinimidyl-carbamate-application-to-acetaminophen-induced-liver-failure
#9
Nicola Gray, Rabiya Zia, Adam King, Vishal C Patel, Julia Wendon, Mark J W McPhail, Muireann Coen, Robert S Plumb, Ian D Wilson, Jeremy K Nicholson
A targeted reversed-phase gradient UPLC-MS/MS assay has been developed for the quantification /monitoring of 66 amino acids and amino-containing compounds in human plasma and serum using precolumn derivatization with 6-aminoquinolyl-N-hydroxysuccinimidyl carbamate (AccQTag Ultra). Derivatization of the target amines required minimal sample preparation and resulted in analytes with excellent chromatographic and mass spectrometric detection properties. The resulting method, which requires only 10 μL of sample, provides the reproducible and robust separation of 66 analytes in 7...
February 7, 2017: Analytical Chemistry
https://www.readbyqxmd.com/read/28188799/structure-elucidation-and-immunological-activity-of-a-novel-glycopeptide-from-mannatide
#10
Yanhong Shen, Mengqi Wu, Zhizhen Xu, Yanfei Wan, Li Zhang, Wenqing Zhang, Wei Xia
A water-soluble glycopeptide, designated as MTW, was isolated and purified from crude mannatide by DEAE-Sepharose. MTW showed one symmetrical peak on HPGPC with an average molecular weight of 2.95×10(4)Da. MTW contained 16 kinds of amino acids and the total amino acid content was 0.95%. The structure of polysaccharide moiety of MTW was elucidated based on monosaccharide composition, methylation analysis, partial acid hydrolysis, IR and 1D/2D NMR spectroscopy. The results showed that MTW was a homogeneous glycopeptide including mannose and glucose with a molar ratio of 2:1...
February 7, 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/28184960/genetic-evidence-of-a-causal-effect-of-insulin-resistance-on-branched-chain-amino-acid-levels
#11
Yuvaraj Mahendran, Anna Jonsson, Christian T Have, Kristine H Allin, Daniel R Witte, Marit E Jørgensen, Niels Grarup, Oluf Pedersen, Tuomas O Kilpeläinen, Torben Hansen
AIMS/HYPOTHESIS: Fasting plasma levels of branched-chain amino acids (BCAAs) are associated with insulin resistance, but it remains unclear whether there is a causal relation between the two. We aimed to disentangle the causal relations by performing a Mendelian randomisation study using genetic variants associated with circulating BCAA levels and insulin resistance as instrumental variables. METHODS: We measured circulating BCAA levels in blood plasma by NMR spectroscopy in 1,321 individuals from the ADDITION-PRO cohort...
February 10, 2017: Diabetologia
https://www.readbyqxmd.com/read/28179968/the-effectiveness-of-a-liver-disease-education-class-for-providing-information-to-patients-and-their-families
#12
Yukio Kadokawa, Kazuhiro Katayama, Kozo Takahashi, Nobuhisa Fukushima, Setsu Tanaka, Yuko Taniguchi, Takatoshi Nawa, Mitsuru Sakakibara, Kazuyoshi Ohkawa
BACKGROUND: We have been conducting liver disease education classes regularly in our hospital for the purpose of providing health information to patients and their families. METHODS: In order to evaluate the effectiveness of these classes, we conducted a questionnaire survey of patients and family members who attended the classes held three times in 2012. The cumulative total number of participants was 80 (49 patients, 26 family members, and five others). The classes focused on the following areas: 1) prevention of hepatic cancer; 2) treatment of hepatic cancer; 3) iron restriction diet for hepatitis C patients; and 4) importance of branched-chain amino acid preparations...
March 2017: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/28179229/exogenous-amino-acids-suppress-glucose-oxidation-and-potentiate-hepatic-glucose-production-in-late-gestation-fetal-sheep
#13
Laura D Brown, Jaden R Kohn, Paul J Rozance, William W Hay, Stephanie R Wesolowski
Acute amino acid (AA) infusion increases AA oxidation rates in normal late gestation fetal sheep. Because fetal oxygen consumption rate does not change with increased AA oxidation, we hypothesized that AA infusion would suppress glucose oxidation pathways and that the additional carbon supply from AA would activate hepatic glucose production. To test this, late gestation fetal sheep were infused intravenously for 3h with saline or exogenous AA (AA). Glucose tracer metabolic studies were performed and skeletal muscle and liver tissues samples were collected...
February 8, 2017: American Journal of Physiology. Regulatory, Integrative and Comparative Physiology
https://www.readbyqxmd.com/read/28178567/defective-branched-chain-amino-acid-catabolism-disrupts-glucose-metabolism-and-sensitizes-the-heart-to-ischemia-reperfusion-injury
#14
Tao Li, Zhen Zhang, Stephen C Kolwicz, Lauren Abell, Nathan D Roe, Maengjo Kim, Bo Zhou, Yang Cao, Julia Ritterhoff, Haiwei Gu, Daniel Raftery, Haipeng Sun, Rong Tian
Elevated levels of branched-chain amino acids (BCAAs) have recently been implicated in the development of cardiovascular and metabolic diseases, but the molecular mechanisms are unknown. In a mouse model of impaired BCAA catabolism (knockout [KO]), we found that chronic accumulation of BCAAs suppressed glucose metabolism and sensitized the heart to ischemic injury. High levels of BCAAs selectively disrupted mitochondrial pyruvate utilization through inhibition of pyruvate dehydrogenase complex (PDH) activity...
February 7, 2017: Cell Metabolism
https://www.readbyqxmd.com/read/28178302/the-2-0-%C3%A3-x-ray-structure-for-yeast-acetohydroxyacid-synthase-provides-new-insights-into-its-cofactor-and-quaternary-structure-requirements
#15
Thierry Lonhienne, Mario D Garcia, James A Fraser, Craig M Williams, Luke W Guddat
Acetohydroxyacid synthase (AHAS) catalyzes the first step of branched-chain amino acid biosynthesis, a pathway essential to the life-cycle of plants and micro-organisms. The catalytic subunit has thiamin diphosphate (ThDP) and flavin adenine dinucleotide (FAD) as indispensable co-factors. A new, high resolution, 2.0 Å crystal structure of Saccharomyces cerevisiae AHAS reveals that the dimer is asymmetric, with the catalytic centres having distinct structures where FAD is trapped in two different conformations indicative of different redox states...
2017: PloS One
https://www.readbyqxmd.com/read/28178201/rett-syndrome-a-focus-on-gut-microbiota
#16
Elisa Borghi, Francesca Borgo, Marco Severgnini, Miriam Nella Savini, Maria Cristina Casiraghi, Aglaia Vignoli
Rett syndrome (RTT) is an X-linked neurodevelopmental disorder affecting 1 in 10,000 live female births. Changes in microbiota composition, as observed in other neurological disorders such as autism spectrum disorders, may account for several symptoms typically associated with RTT. We studied the relationship between disease phenotypes and microbiome by analyzing diet, gut microbiota, and short-chain fatty acid (SCFA) production. We enrolled eight RTT patients and 10 age- and sex-matched healthy women, all without dietary restrictions...
February 7, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28177714/effects-of-high-protein-intake-on-bone-turnover-in-long-term-bed-rest-in-women
#17
Martina Heer, Natalie Baecker, Petra Frings-Meuthen, Sonja Graf, Sara R Zwart, Gianni Biolo, Scott M Smith
PURPOSE: Bed rest (BR) causes bone loss, even in otherwise healthy subjects. Several studies suggest that ambulatory subjects may benefit from high protein intake to stimulate protein synthesis and to maintain muscle mass. However, increasing protein intake above the recommended daily intake without adequate calcium- and potassium intake may increase bone resorption. We hypothesized that a regimen of high protein intake (HiPROT), applied in an isocaloric manner during BR with calcium and potassium intake meeting recommended values, would prevent any effect of BR on bone turnover...
January 19, 2017: Applied Physiology, Nutrition, and Metabolism, Physiologie Appliquée, Nutrition et Métabolisme
https://www.readbyqxmd.com/read/28177706/the-effects-of-acute-branched-chain-amino-acid-supplementation-on-recovery-from-a-single-bout-of-hypertrophy-exercise-in-resistance-trained-athletes
#18
Mark Waldron, Kieran Whelan, Owen Jeffries, Dean Burt, Louis Howe, Stephen David Patterson
This study investigated the effects of acute branched-chain amino acid (BCAA) supplementation on recovery from exercise-induced muscle damage, among experienced resistance-trained athletes. In a double-blind matched-pairs design, 16 resistance-trained participants, routinely performing hypertrophy training, were randomly assigned to a BCAA (n = 8) or placebo (n = 8) group. The BCAAs were administered at a dosage of 0.087 g/kg body mass, with a 2:1:1 ratio of leucine, isoleucine and valine. The participants performed 6 sets of 10 full-squats at 70 % 1RM to induce muscle damage...
January 27, 2017: Applied Physiology, Nutrition, and Metabolism, Physiologie Appliquée, Nutrition et Métabolisme
https://www.readbyqxmd.com/read/28167351/evaluation-of-starch-based-flocculants-for-the-flocculation-of-dissolved-organic-matter-from-textile-dyeing-secondary-wastewater
#19
Hu Wu, Zhouzhou Liu, Aimin Li, Hu Yang
China is a major textile manufacturer in the world; as a result, large quantities of dyeing effluents are generated every year in the country. In this study, the performances of two cationic starch-based flocculants with different chain architectures, i.e., starch-graft-poly[(2-methacryloyloxyethyl) trimethyl ammonium chloride] (STC-g-PDMC) and starch-3-chloro-2-hydroxypropyl trimethyl ammonium chloride (STC-CTA), in flocculating dissolved organic matter (DOM) in dyeing secondary effluents were investigated and compared with that of polyaluminum chloride (PAC)...
January 24, 2017: Chemosphere
https://www.readbyqxmd.com/read/28163025/structural-basis-for-the-transglycosylase-activity-of-a-gh57-type-glycogen-branching-enzyme-from-pyrococcus-horikoshii
#20
Soohui Na, Minjeong Park, Inseong Jo, Jaeho Cha, Nam-Chul Ha
Glycogen branching enzyme (GBE) catalyzes the formation of α-1,6-branching points during glycogenesis by cleaving α-1,4 bonds and making new α-1,6 bonds. Most GBEs belong to the glycoside hydrolase 13 family (GH13), but new GBEs in the GH57 family have been isolated from Archaea. Here, we determined the crystal structure of a GH57 GBE from the hyperthermophilic archaeon Pyrococcus horikoshii (PhGBE) at a resolution of 2.3 Å. PhGBE exhibits both α-1,6-branching activity and endo-α-1,4 hydrolytic activity...
February 3, 2017: Biochemical and Biophysical Research Communications
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