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BCAA metabolism

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https://www.readbyqxmd.com/read/28926411/lower-mitochondrial-dna-and-altered-mitochondrial-fuel-metabolism-in-hiv-exposed-uninfected-infants-in-cameroon
#1
Jennifer Jao, Kathleen M Powis, Brian Kirmse, Chunli Yu, Fanny Epie, Emmanuel Nshom, Elaine J Abrams, Rhoda S Sperling, Derek Leroith, Mitchell E Geffner, Irwin J Kurland, Hélène C F Côté
OBJECTIVE: Evaluate blood mitochondrial DNA (mtDNA) content in HIV/antiretroviral (ARV)-exposed uninfected (HEU) vs. HIV-unexposed uninfected (HUU) infants and investigate differences in mitochondrial-related metabolites by exposure group. DESIGN: We enrolled a prospective cohort of HIV-infected and -uninfected pregnant woman/infant pairs in Cameroon. METHODS: Dried blood spot mtDNA:nuclear DNA ratio was measured by monochrome multiplex qPCR in HEU infants exposed to in utero ARVs and postnatal zidovudine (HEU-Z) or nevirapine (HEU-N), and in HUU infants at 6 weeks of life...
September 18, 2017: AIDS
https://www.readbyqxmd.com/read/28919799/maple-syrup-urine-disease-mechanisms-and-management
#2
REVIEW
Patrick R Blackburn, Jennifer M Gass, Filippo Pinto E Vairo, Kristen M Farnham, Herjot K Atwal, Sarah Macklin, Eric W Klee, Paldeep S Atwal
Maple syrup urine disease (MSUD) is an inborn error of metabolism caused by defects in the branched-chain α-ketoacid dehydrogenase complex, which results in elevations of the branched-chain amino acids (BCAAs) in plasma, α-ketoacids in urine, and production of the pathognomonic disease marker, alloisoleucine. The disorder varies in severity and the clinical spectrum is quite broad with five recognized clinical variants that have no known association with genotype. The classic presentation occurs in the neonatal period with developmental delay, failure to thrive, feeding difficulties, and maple syrup odor in the cerumen and urine, and can lead to irreversible neurological complications, including stereotypical movements, metabolic decompensation, and death if left untreated...
2017: Application of Clinical Genetics
https://www.readbyqxmd.com/read/28919422/over-expression-of-ppar%C3%AE-in-obese-mice-adipose-tissue-improves-insulin-sensitivity
#3
Haruya Takahashi, Kohei Sanada, Hiroyuki Nagai, Yongjia Li, Yumeko Aoki, Takeshi Ara, Shigeto Seno, Hideo Matsuda, Rina Yu, Teruo Kawada, Tsuyoshi Goto
Peroxisome proliferator-activated receptor α (PPARα) is important in the regulation of lipid metabolism and expressed at high levels in the liver. Although PPARα is also expressed in adipose tissue, little is known about the relationship between its activation and the regulation of glucose metabolism. In this study, we developed adipose tissue specific PPARα over-expression (OE) mice. Metabolomics and insulin tolerance tests showed that OE induces branched-chain amino acid (BCAA) profile and improvement of insulin sensitivity...
September 15, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28878358/enoyl-coa-hydratase-1-regulates-mtor-signaling-and-apoptosis-by-sensing-nutrients
#4
Ya-Kun Zhang, Yuan-Yuan Qu, Yan Lin, Xiao-Hui Wu, Hou-Zao Chen, Xu Wang, Kai-Qiang Zhou, Yun Wei, Fushen Guo, Cui-Fang Yao, Xia-Di He, Li-Xia Liu, Chen Yang, Zong-Yuan Guan, Shi-Dong Wang, Jianyuan Zhao, De-Pei Liu, Shi-Min Zhao, Wei Xu
The oncogenic mechanisms of overnutrition, a confirmed independent cancer risk factor, remain poorly understood. Herein, we report that enoyl-CoA hydratase-1 (ECHS1), the enzyme involved in the oxidation of fatty acids (FAs) and branched-chain amino acids (BCAAs), senses nutrients and promotes mTOR activation and apoptotic resistance. Nutrients-promoted acetylation of lys(101) of ECHS1 impedes ECHS1 activity by impairing enoyl-CoA binding, promoting ECHS1 degradation and blocking its mitochondrial translocation through inducing ubiquitination...
September 6, 2017: Nature Communications
https://www.readbyqxmd.com/read/28821850/comparative-metabonomics-of-wenxin-keli-and-verapamil-reveals-differential-roles-of-gluconeogenesis-and-fatty-acid-%C3%AE-oxidation-in-myocardial-injury-protection
#5
Miaomiao Jiang, Qiuying Wang, Jingrui Chen, Yanan Wang, Guanwei Fan, Yan Zhu
Metabonomics/metabolomics is a rapid technology for comprehensive profiling of small molecule metabolites in cells, tissues, or whole organisms, the application of which has led to understanding pathophysiologic mechanisms of cardiometabolic diseases, defining predictive biomarkers for those diseases, and also assessing the efficacious effects of incident drugs. In this study, proton nuclear magnetic resonance (NMR)-based metabonomics was employed to identify the metabolic changes in rat plasma caused by myocardial ischemia-reperfusion injury (MIRI), and to compare the metabolic regulatory differences between traditional Chinese medicine Wenxin Keli (WXKL) and Western medicine verapamil...
August 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28804274/non-invasive-urinary-metabolomic-profiling-discriminates-prostate-cancer-from-benign-prostatic-hyperplasia
#6
Clara Pérez-Rambla, Leonor Puchades-Carrasco, María García-Flores, José Rubio-Briones, José Antonio López-Guerrero, Antonio Pineda-Lucena
INTRODUCTION: Prostate cancer (PCa) is one of the most common malignancies in men worldwide. Serum prostate specific antigen (PSA) level has been extensively used as a biomarker to detect PCa. However, PSA is not cancer-specific and various non-malignant conditions, including benign prostatic hyperplasia (BPH), can cause a rise in PSA blood levels, thus leading to many false positive results. OBJECTIVES: In this study, we evaluated the potential of urinary metabolomic profiling for discriminating PCa from BPH...
2017: Metabolomics: Official Journal of the Metabolomic Society
https://www.readbyqxmd.com/read/28802074/altered-amino-acid-concentrations-in-nafld-impact-of-obesity-and-insulin-resistance
#7
Melania Gaggini, Fabrizia Carli, Chiara Rosso, Emma Buzzigoli, Milena Marietti, Veronica Della Latta, Demetrio Ciociaro, Maria Lorena Abate, Roberto Gambino, Maurizio Cassader, Elisabetta Bugianesi, Amalia Gastaldelli
Plasma concentrations of amino acids (AA), in particular branched chain (BCAA), are often found increased in non-alcoholic fatty liver disease (NAFLD). However, if this is due to increased muscular protein catabolism, obesity and/or increased insulin resistance (IR) or impaired tissue metabolism is not known. Thus, we evaluated a) if subjects with NAFLD non-obese (NAFLD-NO), compared to obese (NAFLD-Ob) display altered plasma AA compared to controls (CT); b) if AA concentrations are associated to IR and liver histology...
August 12, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28768654/effect-of-the-interaction-between-diet-composition-and-the-ppm1k-genetic-variant-on-insulin-resistance-and-%C3%AE-cell-function-markers-during-weight-loss-results-from-the-nutrient-gene-interactions-in-human-obesity-implications-for-dietary-guidelines-nugenob-randomized
#8
RANDOMIZED CONTROLLED TRIAL
Leticia Goni, Lu Qi, Marta Cuervo, Fermín I Milagro, Wim H Saris, Ian A MacDonald, Dominique Langin, Arne Astrup, Peter Arner, Jean-Michel Oppert, Mathilde Svendstrup, Ellen E Blaak, Thorkild Ia Sørensen, Torben Hansen, J Alfredo Martínez
Background: Circulating branched-chain amino acids (BCAAs) and aromatic amino acids (AAAs) have been shown to be associated with insulin resistance and diabetes risk. The common rs1440581 T allele in the protein phosphatase Mg2+/Mn2+ dependent 1K (PPM1K) gene has been related to elevated BCAA concentrations and risk of type 2 diabetes.Objective: In the present study, we tested whether dietary fat and carbohydrate intakes influenced the association between the rs1440581 PPM1K genetic variant and glucose-metabolism traits during weight loss...
September 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28753661/amino-acid-metabolic-signaling-influences-aedes-aegypti-midgut-microbiome-variability
#9
Sarah M Short, Emmanuel F Mongodin, Hannah J MacLeod, Octavio A C Talyuli, George Dimopoulos
The mosquito midgut microbiota has been shown to influence vector competence for multiple human pathogens. The microbiota is highly variable in the field, and the sources of this variability are not well understood, which limits our ability to understand or predict its effects on pathogen transmission. In this work, we report significant variation in female adult midgut bacterial load between strains of A. aegypti which vary in their susceptibility to dengue virus. Composition of the midgut microbiome was similar overall between the strains, with 81-92% of reads coming from the same five bacterial families, though we did detect differences in the presence of some bacterial families including Flavobacteriaceae and Entobacteriaceae...
July 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28752051/repletion-of-branched-chain-amino-acids-reverses-mtorc1-signaling-but-not-improved-metabolism-during-dietary-protein-dilution
#10
Adriano Maida, Jessica S K Chan, Kim A Sjøberg, Annika Zota, Dieter Schmoll, Bente Kiens, Stephan Herzig, Adam J Rose
OBJECTIVE: Dietary protein dilution (PD) has been associated with metabolic advantages such as improved glucose homeostasis and increased energy expenditure. This phenotype involves liver-induced release of FGF21 in response to amino acid insufficiency; however, it has remained unclear whether dietary dilution of specific amino acids (AAs) is also required. Circulating branched chain amino acids (BCAAs) are sensitive to protein intake, elevated in the serum of obese humans and mice and thought to promote insulin resistance...
August 2017: Molecular Metabolism
https://www.readbyqxmd.com/read/28750610/an-exploratory-study-on-the-efficacy-and-safety-of-a-bcaa-preparation-used-in-combination-with-cardiac-rehabilitation-for-patients-with-chronic-heart-failure
#11
Munenori Takata, Eisuke Amiya, Masafumi Watanabe, Yumiko Hosoya, Atsuko Nakayama, Takayuki Fujiwara, Masanobu Taya, Gaku Oguri, Kanako Hyodo, Naoko Takayama, Nami Takano, Tomoe Mashiko, Yukari Uemura, Issei Komuro
BACKGROUND: Sarcopenia is generally complicated with patients with chronic heart failure (CHF) and its presence negatively affects the course of heart failure, however effective nutritional intervention had not been elucidated yet. The primary objective of this study is to explore whether the addition of a branched-chain amino acid (BCAA) preparation for cardiac rehabilitation (CR) of patients with CHF further improves cardiopulmonary functions, skeletal muscle functions, and metabolism in comparison with conventional CR...
July 27, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28744267/the-staphylococcus-aureus-%C3%AE-acetolactate-synthase-als-confers-resistance-to-nitrosative-stress
#12
Sandra M Carvalho, Anne de Jong, Tomas G Kloosterman, Oscar P Kuipers, Lígia M Saraiva
Staphylococcus aureus is a worldwide pathogen that colonizes the human nasal cavity and is a major cause of respiratory and cutaneous infections. In the nasal cavity, S. aureus thrives with high concentrations of nitric oxide (NO) produced by the innate immune effectors and has available for growth slow-metabolizing free hexoses, such as galactose. Here, we have used deep sequencing transcriptomic analysis (RNA-Seq) and (1)H-NMR to uncover how S. aureus grown on galactose, a major carbon source present in the nasopharynx, survives the deleterious action of NO...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28741271/supplementation-with-branched-chain-amino-acids-ameliorates-hypoalbuminemia-prevents-sarcopenia-and-reduces-fat-accumulation-in-the-skeletal-muscles-of-patients-with-liver-cirrhosis
#13
Yoichiro Kitajima, Hirokazu Takahashi, Takumi Akiyama, Kenichiro Murayama, Shinji Iwane, Takuya Kuwashiro, Kenichi Tanaka, Seiji Kawazoe, Naofumi Ono, Takahisa Eguchi, Keizo Anzai, Yuichiro Eguchi
BACKGROUND: Liver cirrhosis induces marked metabolic disorders, protein-energy malnutrition, and sarcopenia. The objective of the study reported here was to investigate the effects of dietary branched-chain amino acids (BCAAs) on systemic glucose metabolism, skeletal muscle, and prognosis of patients with liver cirrhosis. METHODS: Japanese patients with liver cirrhosis (n = 21) were enrolled into a longitudinal study in which their diets were supplemented with BCAAs...
July 24, 2017: Journal of Gastroenterology
https://www.readbyqxmd.com/read/28735462/effect-of-decreased-bcaa-synthesis-through-disruption-of-ilvc-gene-on-the-virulence-of-streptococcus-pneumoniae
#14
Gyu-Lee Kim, Seungyeop Lee, Truc Thanh Luong, Cuong Thach Nguyen, Sang-Sang Park, Suhkneung Pyo, Dong-Kwon Rhee
Streptococcus pneumoniae (pneumococcus) is responsible for significant morbidity and mortality worldwide. It causes a variety of life-threatening infections such as pneumonia, bacteremia, and meningitis. In bacterial physiology, the metabolic pathway of branched-chain amino acids (BCAAs) plays an important role in virulence. Nonetheless, the function of IlvC, one of the enzymes involved in the biosynthesis of BCAAs, in S. pneumoniae remains unclear. Here, we demonstrated that downregulation of BCAA biosynthesis by ilvC ablation can diminish BCAA concentration and expression of pneumolysin (Ply) and LytA, and subsequently attenuate virulence...
July 22, 2017: Archives of Pharmacal Research
https://www.readbyqxmd.com/read/28728397/effects-of-dietary-valine-lysine-ratio-on-the-performance-amino-acid-composition-of-tissues-and-mrna-expression-of-genes-involved-in-branched-chain-amino-acid-metabolism-of-weaned-piglets
#15
Yetong Xu, Xiaokang Ma, Chunli Wang, Mingfeng Yuan, Xiangshu Piao
Objective: The goal of this study was to investigate the effects of dietary standard ileal digestible (SID) valine:lysine ratios on performance, intestinal morphology, amino acids of liver and muscle, plasma indices and mRNA expression of branched-chain amino acid (BCAA) metabolism enzymes. Methods: A total of 144 crossbred pigs (Duroc × Landrace × Large White) weaned at 28 ± 4 days of age (8.79 ± 0.02 kg body weight) were randomly allotted to 1 of 4 diets formulated to provide SID valine: lysine ratios of 50, 60, 70 or 80%...
May 14, 2017: Asian-Australasian Journal of Animal Sciences
https://www.readbyqxmd.com/read/28719620/endurance-performance-and-energy-metabolism-during-exercise-in-mice-with-a-muscle-specific-defect-in-the-control-of-branched-chain-amino-acid-catabolism
#16
Minjun Xu, Yasuyuki Kitaura, Takuya Ishikawa, Yoshihiro Kadota, Chihaya Terai, Daichi Shindo, Takashi Morioka, Miki Ota, Yukako Morishita, Kengo Ishihara, Yoshiharu Shimomura
It is known that the catabolism of branched-chain amino acids (BCAAs) in skeletal muscle is suppressed under normal and sedentary conditions but is promoted by exercise. BCAA catabolism in muscle tissues is regulated by the branched-chain α-keto acid (BCKA) dehydrogenase complex, which is inactivated by phosphorylation by BCKA dehydrogenase kinase (BDK). In the present study, we used muscle-specific BDK deficient mice (BDK-mKO mice) to examine the effect of uncontrolled BCAA catabolism on endurance exercise performance and skeletal muscle energy metabolism...
2017: PloS One
https://www.readbyqxmd.com/read/28717019/3-hydroxyisobutyryl-coa-hydrolase-involved-in-isoleucine-catabolism-regulates-triacylglycerol-accumulation-in-phaeodactylum-tricornutum
#17
Yufang Pan, Juan Yang, Yangmin Gong, Xiaolong Li, Hanhua Hu
Since methylmalonyl-CoA epimerase appears to be absent in the majority of photosynthetic organisms, including diatoms, (S)-methylmalonyl-CoA, the intermediate of isoleucine (Ile) catabolism, cannot be metabolized to (R)-methylmalonyl-CoA then to succinyl-CoA. In this study, propionyl-CoA carboxylase (PCC) RNAi silenced strains and 3-hydroxyisobutyryl-CoA hydrolase (HIBCH) overexpression strains were constructed to elucidate the Ile degradation pathway and its influence on lipid accumulation in Phaeodactylum tricornutum based on growth, neutral lipid content and metabolite profile analysis...
September 5, 2017: Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences
https://www.readbyqxmd.com/read/28713581/short-term-elevation-in-dietary-protein-intake-does-not-worsen-insulin-resistance-or-lipids-in-older-adults-with-metabolic-syndrome-a-randomized-controlled-trial
#18
Il-Young Kim, Scott E Schutzler, Gohar Azhar, Robert R Wolfe, Arny A Ferrando, Robert H Coker
BACKGROUND: There is a great deal of controversy as to whether higher protein intake improves or worsens insulin sensitivity in humans. The purpose of the study was to determine the influence of a short-term elevation in dietary protein on hepatic and peripheral insulin sensitivity in twelve older subjects (51-70 yrs) with metabolic syndrome. METHODS: Individuals were randomly assigned to one of the dietary groups: recommended protein intake (RPI, 10% of daily calorie intake) or elevated protein intake (EPI, 20% of daily calorie intake) for 4 weeks...
2017: BMC Nutrition
https://www.readbyqxmd.com/read/28705827/3-hydroxyisobutyrate-dehydrogenase-is-involved-in-both-valine-and-isoleucine-degradation-in-arabidopsis-thaliana
#19
Peter Schertl, Lennart Danne, Hans-Peter Braun
In plants, amino acid catabolism is especially relevant in metabolic stress situations (e.g. limited carbohydrate availability during extended darkness). Under these conditions, amino acids are used as alternative substrates for respiration. Complete oxidation of the branched-chain amino acids (BCAAs) leucine, isoleucine (Ile), and valine (Val) in the mitochondria efficiently allows the formation of ATP by oxidative phosphorylation. However, the metabolic pathways for BCAA breakdown are largely unknown so far in plants...
September 2017: Plant Physiology
https://www.readbyqxmd.com/read/28699638/bcat1-controls-metabolic-reprogramming-in-activated-human-macrophages-and-is-associated-with-inflammatory-diseases
#20
Adonia E Papathanassiu, Jeong-Hun Ko, Martha Imprialou, Marta Bagnati, Prashant K Srivastava, Hong A Vu, Danilo Cucchi, Stephen P McAdoo, Elitsa A Ananieva, Claudio Mauro, Jacques Behmoaras
Branched-chain aminotransferases (BCAT) are enzymes that initiate the catabolism of branched-chain amino acids (BCAA), such as leucine, thereby providing macromolecule precursors; however, the function of BCATs in macrophages is unknown. Here we show that BCAT1 is the predominant BCAT isoform in human primary macrophages. We identify ERG240 as a leucine analogue that blocks BCAT1 activity. Selective inhibition of BCAT1 activity results in decreased oxygen consumption and glycolysis. This decrease is associated with reduced IRG1 levels and itaconate synthesis, suggesting involvement of BCAA catabolism through the IRG1/itaconate axis within the tricarboxylic acid cycle in activated macrophages...
July 12, 2017: Nature Communications
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