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Branched-chain amino acid

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https://www.readbyqxmd.com/read/28238968/changes-in-macroautophagy-chaperone-mediated-autophagy-and-mitochondrial-metabolism-in-murine-skeletal-and-cardiac-muscle-during-aging
#1
Jin Zhou, Shu Yun Chong, Andrea Lim, Brijesh K Singh, Rohit A Sinha, Adam B Salmon, Paul M Yen
Aging causes a general decline in cellular metabolic activity, and function in different tissues and whole body homeostasis. However, the understanding about the metabolomic and autophagy changes in skeletal muscle and heart during aging is still limited. We thus examined markers for macroautophagy, chaperone-mediated autophagy (CMA), mitochondrial quality control, as well as cellular metabolites in skeletal and cardiac muscle from young (5 months old) and aged (27 months old) mice. We found decreased autophagic degradation of p62 and increased ubiquitinated proteins in both tissues from aged mice, suggesting a decline in macroautophagy during aging...
February 26, 2017: Aging
https://www.readbyqxmd.com/read/28238072/gill-damage-and-neurotoxicity-of-ammonia-nitrogen-on-the-clam-ruditapes-philippinarum
#2
Ming Cong, Huifeng Wu, Haiping Yang, Jianmin Zhao, Jiasen Lv
Ammonia nitrogen has been a potential menace to aquatic animals along the coastline of China. Presently, the toxicological effects of ammonia nitrogen were mainly concentrated on fishes, while little attention has been paid to molluscs. In this study, the clam Ruditapes philippinarum was used as the target animal to investigate the toxic effects of ammonia nitrogen. Our results showed that ammonia exposure could significantly reduce the integrity of lysosomes in a dose-dependent manner. Metabolite analysis revealed that exposure doses and duration time of ammonia nitrogen could affect the variation profiles of gill metabolites...
February 25, 2017: Ecotoxicology
https://www.readbyqxmd.com/read/28236608/normal-weight-but-low-muscle-mass-and-abdominally-obese-implications-for-the-cardiometabolic-risk-profile-in-chronic-obstructive-pulmonary-disease
#3
Rosanne J H C G Beijers, Coby van de Bool, Bram van den Borst, Frits M E Franssen, Emiel F M Wouters, Annemie M W J Schols
BACKGROUND: It is well established that low muscle mass affects physical performance in chronic obstructive pulmonary disease (COPD). We hypothesize that combined low muscle mass and abdominal obesity may also adversely influence the cardiometabolic risk profile in COPD, even in those with normal weight. The cardiometabolic risk profile and the responsiveness to 4 months high-intensity exercise training was assessed in normal-weight patients with COPD with low muscle mass stratified by abdominal obesity...
February 21, 2017: Journal of the American Medical Directors Association
https://www.readbyqxmd.com/read/28235484/branched-chain-amino-acid-transaminase-1-bcat1-promotes-the-growth-of-breast-cancer-cells-through-improving-mtor-mediated-mitochondrial-biogenesis-and-function
#4
Ling Zhang, Junqing Han
Branched-chain amino acids (BCAAs) are important nutrient signals that have direct and indirect effects. BCAA catabolism is a conserved regulator of physiological aging and participates in diverse physiological and pathological processes, including carcinoma development. The roles of BCAA catabolism in human breast cancer remains unknown. Here we provide evidence that BCAA catabolism is involved in human breast cancer. The plasma and tissue levels of BCAAs are increased in breast cancer, which is accompanied by the elevated expression of the catabolic enzymes, including branched-chain amino acid transaminase 1 (BCAT1)...
February 21, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28230078/unravelling-the-metabolic-impact-of-sbs-associated-microbial-dysbiosis-insights-from-the-piglet-short-bowel-syndrome-model
#5
Prue M Pereira-Fantini, Sean G Byars, James Pitt, Susan Lapthorne, Fiona Fouhy, Paul D Cotter, Julie E Bines
Liver disease is a major source of morbidity and mortality in children with short bowel syndrome (SBS). SBS-associated microbial dysbiosis has recently been implicated in the development of SBS-associated liver disease (SBS-ALD), however the pathological implications of this association have not been explored. In this study high-throughput sequencing of colonic content from the well-validated piglet SBS-ALD model was examined to determine alterations in microbial communities, and concurrent metabolic alterations identified in urine samples via targeted mass spectrometry approaches (GC-MS, LC-MS, FIA-MS) further uncovered impacts of microbial disturbance on metabolic outcomes in SBS-ALD...
February 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28223686/metabolic-profiling-of-presymptomatic-huntington-s-disease-sheep-reveals-novel-biomarkers
#6
Debra J Skene, Benita Middleton, Cara K Fraser, Jeroen L A Pennings, Timothy R Kuchel, Skye R Rudiger, C Simon Bawden, A Jennifer Morton
The pronounced cachexia (unexplained wasting) seen in Huntington's disease (HD) patients suggests that metabolic dysregulation plays a role in HD pathogenesis, although evidence of metabolic abnormalities in HD patients is inconsistent. We performed metabolic profiling of plasma from presymptomatic HD transgenic and control sheep. Metabolites were quantified in sequential plasma samples taken over a 25 h period using a targeted LC/MS metabolomics approach. Significant changes with respect to genotype were observed in 89/130 identified metabolites, including sphingolipids, biogenic amines, amino acids and urea...
February 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28219950/nutritional-modulation-of-mouse-and-human-liver-bud-growth-through-a-branched-amino-acid-metabolism
#7
Hiroyuki Koike, Ran-Ran Zhang, Yasuharu Ueno, Keisuke Sekine, Yun-Wen Zheng, Takanori Takebe, Hideki Taniguchi
Liver bud progenitors experience a transient amplification during early organ growth phase, yet the responsible mechanism was not fully understood. Collective evidence highlighted the specific requirements in stem cell metabolism for expanding organ progenitors during organogenesis and regeneration. Here, transcriptome analyses showed progenitors of mouse and human liver bud growth stage specifically expressed branched chain aminotransferase1 gene, a known breakdown enzyme of branched-chain amino acid (BCAA) for energy generation...
February 20, 2017: Development
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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