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https://www.readbyqxmd.com/read/28324240/neurocognitive-profiles-in-msud-school-age-patients
#1
Juliette Bouchereau, Julie Leduc-Leballeur, Samia Pichard, Apolline Imbard, Jean-François Benoist, Marie-Thérèse Abi Warde, Jean-Baptiste Arnoux, Valérie Barbier, Anaïs Brassier, Pierre Broué, Aline Cano, Brigitte Chabrol, Gilles Damon, Claire Gay, Isabelle Guillain, Florence Habarou, Delphine Lamireau, Chris Ottolenghi, Laetitia Paermentier, Frédérique Sabourdy, Guy Touati, Hélène Ogier de Baulny, Pascale de Lonlay, Manuel Schiff
Maple syrup urine disease (MSUD), an inborn error of amino acids catabolism is characterized by accumulation of branched chain amino acids (BCAAs) leucine, isoleucine, valine and their corresponding alpha-ketoacids. Impact on the cognitive development has been reported historically, with developmental delays of varying degree. Currently, earlier diagnosis and improved management allow a better neurodevelopment, without requirement of special education. However, specific impairments can be observed, and so far, results of detailed neurocognitive assessments are not available...
March 21, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28323986/chronic-intranasal-insulin-does-not-affect-hepatic-lipids-but-lowers-circulating-bcaas-in-healthy-male-subjects
#2
Thomas Scherer, Peter Wolf, Sabina Smajis, Melania Gaggini, Martina Hackl, Amalia Gastaldelli, Peter Klimek, Elisa Einwallner, Rodrig Marculescu, Anton Luger, Clemens Fürnsinn, Siegfried Trattnig, Christoph Buettner, Martin Krššák, Michael Krebs
Context: Non-alcoholic fatty liver disease and elevated circulating branched-chain amino acids (BCAAs) are common characteristics of obesity and type 2 diabetes. In rodents, brain insulin signaling controls both hepatic triglyceride secretion and BCAA catabolism. Whether brain insulin signaling controls similar metabolic pathways in humans is unknown. Objective: Here we assessed if intranasal insulin, a method to preferentially deliver insulin to the central nervous system, is able to modulate hepatic lipid content and plasma BCAAs in humans...
January 16, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28296401/effects-of-low-protein-diets-supplemented-with-branched-chain-amino-acids-on-lipid-metabolism-in-white-adipose-tissue-of-piglets
#3
Yinghui Li, Hongkui Wei, Fengna Li, Yehui Duan, Qiuping Guo, Yulong Yin
This study was to evaluate the effect of branched-chain amino acids (BCAA) supplementation in low-protein diets on lipid metabolism in dorsal subcutaneous adipose (DSA), abdominal subcutaneous adipose (ASA), and perirenal adipose (PRA) tissues. A total of 24 piglets were allotted to 4 treatments, and each group fed the adequate protein (AP) diet, low-protein (LP) diet, LP diet supplemented with BCAA (LP + B), or LP diet supplemented with twice BCAA (LP + 2B). Serum concentrations of leptin in the BCAA supplemented treatments were higher (P < 0...
March 15, 2017: Journal of Agricultural and Food Chemistry
https://www.readbyqxmd.com/read/28285648/branched-chain-amino-acids-alleviate-hepatic-steatosis-and-liver-injury-in-choline-deficient-high-fat-diet-induced-nash-mice
#4
Takashi Honda, Masatoshi Ishigami, Fangqiong Luo, Ma Lingyun, Yoji Ishizu, Teiji Kuzuya, Kazuhiko Hayashi, Isao Nakano, Tetsuya Ishikawa, Guo-Gang Feng, Yoshiaki Katano, Tomoya Kohama, Yasuyuki Kitaura, Yoshiharu Shimomura, Hidemi Goto, Yoshiki Hirooka
BACKGROUND: For successful treatment for nonalcoholic steatohepatitis (NASH), it may be important to treat the individual causative factors. At present, however, there is no established treatment for this disease. Branched-chain amino acids (BCAAs) have been used to treat patients with decompensated cirrhosis. AIM: In order to elucidate the mechanisms responsible for the effects of BCAAs on hepatic steatosis and disease progression, we investigated the effects of BCAA supplementation in mice fed a choline-deficient high-fat diet (CDHF), which induces NASH...
April 2017: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/28271897/branched-chain-amino-acids-as-predictors-for-individual-differences-of-cisplatin-nephrotoxicity-in-rats-a-pharmacometabonomics-study
#5
Pei Zhang, Wei Li, Jiaqing Chen, Ruiting Li, Zunjian Zhang, Yin Huang, Fengguo Xu
Nephrotoxicity is the dose-limiting adverse effect of cisplatin with large individual differences. Up to now, little has been done on how to recognize and predict the individual differences in either preclinical or clinical research. In the present study, important postdose indicators were screened out first and integrated into a grouping factor, according to which rats were recognized as lowly or highly sensitive individuals. Then, mass-spectrometry-based untargeted metabolomics approach was performed to dissect the metabolic differences in predose serum of the two groups...
March 17, 2017: Journal of Proteome Research
https://www.readbyqxmd.com/read/28266085/branched-chain-amino-acid-ratios-in-low-protein-diets-regulate-the-free-amino-acid-profile-and-the-expression-of-hepatic-fatty-acid-metabolism-related-genes-in-growing-pigs
#6
Y H Duan, F N Li, C Y Wen, W L Wang, Q P Guo, Y H Li, Y L Yin
Liver metabolism is affected by nutrients. The aim of this study was to explore the effects of low-protein diets (17% crude protein, CP) supplemented with branched-chain amino acids (BCAAs), including leucine (Leu), isoleucine (Ile) and valine (Val), on hepatic amino acid profile and lipid metabolism in growing pigs. The ratio of Leu : Ile : Val in all groups was 1 : 0.51 : 0.63 (20% crude protein, CP), 1 : 1 : 1 (17% CP), 1 : 0.75 : 0.75 (17% CP), 1 : 0.51 : 0.63 (17% CP) and 1 : 0.25 : 0...
March 6, 2017: Journal of Animal Physiology and Animal Nutrition
https://www.readbyqxmd.com/read/28265960/impact-of-bariatric-surgery-on-metabolic-and-gut-microbiota-profile-a-systematic-review-and-meta-analysis
#7
REVIEW
Dimitrios E Magouliotis, Vasiliki S Tasiopoulou, Eleni Sioka, Christina Chatedaki, Dimitrios Zacharoulis
We aim to review the available literature on obese patients treated with bariatric procedures, in order to assess their effect on the metabolic and gut microbiota profiles. A systematic literature search was performed in PubMed, Cochrane library, and Scopus databases, in accordance with the PRISMA guidelines. Twenty-two studies (562 patients) met the inclusion criteria. This study points to significant amelioration of postoperative levels of glucose, insulin, triglycerides, total cholesterol, LDL, HDL, HOMA-IR, food intake, and diabetes remission...
March 6, 2017: Obesity Surgery
https://www.readbyqxmd.com/read/28238968/changes-in-macroautophagy-chaperone-mediated-autophagy-and-mitochondrial-metabolism-in-murine-skeletal-and-cardiac-muscle-during-aging
#8
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/28235484/branched-chain-amino-acid-transaminase-1-bcat1-promotes-the-growth-of-breast-cancer-cells-through-improving-mtor-mediated-mitochondrial-biogenesis-and-function
#9
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 22, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28219950/nutritional-modulation-of-mouse-and-human-liver-bud-growth-through-a-branched-amino-acid-metabolism
#10
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
#11
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/28199774/sarcopenia-predicts-minimal-hepatic-encephalopathy-in-patients-with-liver-cirrhosis
#12
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/28197878/two-homozygous-mutations-in-the-exon-5-of-bckdhb-gene-that-may-cause-the-classic-form-of-maple-syrup-urine-disease
#13
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/28184960/genetic-evidence-of-a-causal-effect-of-insulin-resistance-on-branched-chain-amino-acid-levels
#14
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/28178567/defective-branched-chain-amino-acid-catabolism-disrupts-glucose-metabolism-and-sensitizes-the-heart-to-ischemia-reperfusion-injury
#15
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/28177714/effects-of-high-protein-intake-on-bone-turnover-in-long-term-bed-rest-in-women
#16
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
#17
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/28159840/the-role-of-a-fad-cofactor-in-the-regulation-of-acetohydroxyacid-synthase-by-redox-signaling-molecules
#18
Thierry Lonhienne, Mario D Garcia, Luke W Guddat
Acetohydroxyacid synthase (AHAS) catalyzes the first step of branched-chain amino acid (BCAA) biosynthesis, a pathway essential to the lifecycle of plants and microorganisms. This enzyme is of high interest because its inhibition is at the base of the exceptional potency of herbicides and potentially a target for the discovery of new antimicrobial drugs. The enzyme has conserved attributes from its predicted ancestor, pyruvate oxidase, such as a ubiquinone-binding site and the requirement for FAD as cofactor...
February 3, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28145418/chronic-dietary-exposure-to-branched-chain-amino-acids-impairs-glucose-disposal-in-vegans-but-not-in-omnivores
#19
J Gojda, L Rossmeislová, R Straková, J Tůmová, M Elkalaf, M Jaček, P Tůma, J Potočková, E Krauzová, P Waldauf, J Trnka, V Štich, M Anděl
BACKGROUND/OBJECTIVES: Branched chain amino acids (BCAA) are among nutrients strongly linked with insulin sensitivity (IS) measures. We investigated the effects of a chronic increase of BCAA intake on IS in two groups of healthy subjects differing in their basal consumption of BCAA, that is, vegans and omnivores. SUBJECTS/METHODS: Eight vegans and eight matched omnivores (five men and three women in each group) received 15 g (women) or 20 g (men) of BCAA daily for 3 months...
February 1, 2017: European Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28130304/ccpa-and-cody-coordinate-acetate-metabolism-in-streptococcus-mutans
#20
Jeong Nam Kim, Robert A Burne
In the dental caries pathogen Streptococcus mutans, phosphotransacetylase (Pta) and acetate kinase (Ack) convert pyruvate into acetate with the concomitant generation of ATP. The genes for this pathway are tightly regulated by multiple environmental and intracellular inputs, but the basis for differential expression of the genes for Pta and Ack in S. mutans had not been investigated. Here, we show that inactivation in S. mutans of ccpA or codY reduced the activity of the ackA promoter, whereas a ccpA mutant displayed elevated pta promoter activity...
January 27, 2017: Applied and Environmental Microbiology
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