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phenylalanine metabolism

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https://www.readbyqxmd.com/read/28446738/work-activity-and-phenylalanine-levels-in-a-population-of-young-adults-with-classic-pku
#1
Michele Augusto Riva, Fabiana Madotto, Massimo Turato, Elisabetta Salvatici, Silvia Indovina, Marcello Giovannini, Enrica Riva, Giancarlo Cesana
BACKGROUND: Phenylketonuria (PKU) is an inborn error of metabolism characterized by increased blood concentrations of phenylalanine (Phe). OBJECTIVES: The aim of the present study was to assess the association between the metabolic compliance of adult patients affected by classic PKU and the characteristics of their present and past occupations. METHODS: The study population consisted of working adults, affected by classic PKU, and following a dietary treatment...
April 21, 2017: La Medicina del Lavoro
https://www.readbyqxmd.com/read/28444480/model-based-biotechnological-potential-analysis-of-kluyveromyces-marxianus-central-metabolism
#2
A Pentjuss, E Stalidzans, J Liepins, A Kokina, J Martynova, P Zikmanis, I Mozga, R Scherbaka, H Hartman, M G Poolman, D A Fell, A Vigants
The non-conventional yeast Kluyveromyces marxianus is an emerging industrial producer for many biotechnological processes. Here, we show the application of a biomass-linked stoichiometric model of central metabolism that is experimentally validated, and mass and charge balanced for assessing the carbon conversion efficiency of wild type and modified K. marxianus. Pairs of substrates (lactose, glucose, inulin, xylose) and products (ethanol, acetate, lactate, glycerol, ethyl acetate, succinate, glutamate, phenylethanol and phenylalanine) are examined by various modelling and optimisation methods...
April 25, 2017: Journal of Industrial Microbiology & Biotechnology
https://www.readbyqxmd.com/read/28442765/the-gpr139-reference-agonists-1a-and-7c-and-tryptophan-and-phenylalanine-share-a-common-binding-site
#3
Anne Cathrine Nøhr, Willem Jespers, Mohamed A Shehata, Leonard Floryan, Vignir Isberg, Kirsten Bayer Andersen, Johan Åqvist, Hugo Gutiérrez-de-Terán, Hans Bräuner-Osborne, David E Gloriam
GPR139 is an orphan G protein-coupled receptor expressed in the brain, in particular in the habenula, hypothalamus and striatum. It has therefore been suggested that GPR139 is a possible target for metabolic disorders and Parkinson's disease. Several surrogate agonist series have been published for GPR139. Two series published by Shi et al. and Dvorak et al. included agonists 1a and 7c respectively, with potencies in the ten-nanomolar range. Furthermore, Isberg et al. and Liu et al. have previously shown that tryptophan (Trp) and phenylalanine (Phe) can activate GPR139 in the hundred-micromolar range...
April 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28441769/uva-uvb-and-uvc-light-enhances-the-biosynthesis-of-phenolic-antioxidants-in-fresh-cut-carrot-through-a-synergistic-effect-with-wounding
#4
Bernadeth B Surjadinata, Daniel A Jacobo-Velázquez, Luis Cisneros-Zevallos
Previously, we found that phenolic content and antioxidant capacity (AOX) in carrots increased with wounding intensity. It was also reported that UV radiation may trigger the phenylpropanoid metabolism in plant tissues. Here, we determined the combined effect of wounding intensity and UV radiation on phenolic compounds, AOX, and the phenylalanine ammonia-lyase (PAL) activity of carrots. Accordingly, phenolic content, AOX, and PAL activity increased in cut carrots with the duration of UVC radiation, whereas whole carrots showed no increase...
April 24, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/28440547/development-of-lc-qtof-ms-method-for-human-lung-tissue-fingerprinting-a-preliminary-application-to-non-small-cell-lung-cancer
#5
Michal Ciborowski, Joanna Kisluk, Karolina Pietrowska, Paulina Samczuk, Ewa Parfieniuk, Tomasz Kowalczyk, Miroslaw Kozlowski, Adam Kretowski, Jacek Niklinski
The major histologic subtypes of non-small cell lung cancer (NSCLC) include adenocarcinoma (ADC), squamous cell lung carcinoma (SCC), and large-cell carcinoma (LCC). Clinical trials of targeted agents and newer chemotherapy agents yielded differences in outcomes according to histologic subgroups providing a rationale for histology-based treatment in NSCLC. Currently NSCLC subtyping is performed based on histopathological examinations and immunohistochemistry. However available methods leave about 10% of NSCLC cases as not otherwise specified...
April 25, 2017: Electrophoresis
https://www.readbyqxmd.com/read/28437605/aqueously-released-graphene-oxide-embedded-in-epoxy-resin-exhibits-different-characteristics-and-phytotoxicity-of-chlorella-vulgaris-from-the-pristine-form
#6
Xiangang Hu, Weilu Kang, Li Mu
The environmental release of nanoparticles is attracting increasing attention. Graphene oxide (GO) embedded in epoxy resin (ER) is a popular composite that has been used in various fields, but the environmental release of GO-ER composites and the effects on organisms in the environment remain unknown. The present work found that GO-ER composites in water for 2-7 days resulted in the release of 0.3-2.1% GO-ER at nanoscale (2-3 nm thickness and approximately 70-130 nm lateral length). Interestingly, pristine GO quenched 30-45% hydroxyl and 12% nitroxide free radicals, whereas this capacity was not observed for the released particles from GO-ER...
April 24, 2017: Environmental Science & Technology
https://www.readbyqxmd.com/read/28436122/metabolic-engineering-of-escherichia-coli-for-production-of-2-phenylethylacetate-from-l-phenylalanine
#7
Daoyi Guo, Lihua Zhang, Hong Pan, Xun Li
In order to meet the need of consumer preferences for natural flavor compounds, microbial synthesis method has become a very attractive alternative to the chemical production. The 2-phenylethanol (2-PE) and its ester 2-phenylethylacetate (2-PEAc) are two extremely important flavor compounds with a rose-like odor. In recent years, Escherichia coli and yeast have been metabolically engineered to produce 2-PE. However, a metabolic engineering approach for 2-PEAc production is rare. Here, we designed and expressed a 2-PEAc biosynthetic pathway in E...
April 24, 2017: MicrobiologyOpen
https://www.readbyqxmd.com/read/28433594/favouring-no-over-h2o2-production-will-increase-pb-tolerance-in-prosopis-farcta-via-altered-primary-metabolism
#8
Somaieh Zafari, Mohsen Sharifi, Luis A J Mur, Najmeh Ahmadian Chashmi
Reactive oxygen species (ROS) and nitric oxide (NO) are known in triggering defense functions to detoxify heavy metal stresses. To investigate the relevance of ROS production, Pb treatment (400µM) alone and in combination with 400µM sodium ascorbate (Asc: as H2O2 scavenger) were given to hydroponically grown Prosopis farcta seedlings over a time course of 72h. Data presented here indicate that, the low extent of H2O2 due to scavenging by ascorbate, together with high level of NO improved Pb+Asc- treated Prosopis growth...
April 20, 2017: Ecotoxicology and Environmental Safety
https://www.readbyqxmd.com/read/28422049/regulation-of-anthocyanin-biosynthesis-in-purple-leaves-of-zijuan-tea-camellia-sinensis-var-kitamura
#9
Lingxia Wang, Dezhuo Pan, Meng Liang, Yakubu Saddeeq Abubakar, Jian Li, Jinke Lin, Shipin Chen, Wei Chen
Plant anthocyanin biosynthesis is well understood, but the regulatory mechanism in purple foliage tea remains unclear. Using isobaric tag for relative and absolute quantification (iTRAQ), 815 differential proteins were identified in the leaves of Zijuan tea, among which 20 were associated with the regulation of anthocyanin metabolism. We found that the abundances of anthocyanin synthesis-related enzymes such as chalcone synthase, chalcone isomerase, dihydroflavonol 4-reductase and anthocyanin synthetase, as well as anthocyanin accumulation-related UDP-glucosyl transferase and ATP-binding cassette (ABC) transporters in the purple leaves were all significantly higher than those in the green leaves...
April 19, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28420061/metabolic-profiling-reveals-new-serum-biomarkers-of-lupus-nephritis
#10
J Li, X-W Xie, H Zhou, B Wang, M-J Zhang, F-Y Tang
Metabolomics has been applied to explore altered metabolite profiles in disease and identify unique metabolic signatures specific to certain pathologies. The aim of the current study is to characterize the metabolic profile of patients diagnosed with lupus nephritis (LN) and explore new insights into underlying disease processes. A metabolomic approach using ultra-high-performance liquid chromatography coupled with high-resolution mass spectrometry (UPLC-HRMS) was developed in serum samples from 32 LN patients, 30 idiopathic nephrotic syndrome (INS) patients and 28 healthy controls (HCs)...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28411543/the-disorder-metabolic-profiling-in-kidney-and-spleen-of-mice-induced-by-mycotoxins-deoxynivalenol-through-gas-chromatography-mass-spectrometry
#11
Jian Ji, Pei Zhu, Fangchao Cui, Fuwei Pi, Yinzhi Zhang, Xiulan Sun
Gas chromatography mass spectrometry (GC-MS) based metabolomics strategy was implemented for the metabolites detection in kidney and spleen samples of mice, which were treated with 2 mg kg(-1) deoxynivalenol (DON), through intragastric administration for three weeks, for studying the toxicity of DON on the metabolic profiling in kidney and spleen. The spectrum was deconvoluted, aligned and identified with MS DIAL, equipped with Fiehn library. And the data matrix was processed with univariate analysis and multivariate analysis for selection of metabolites with VIP >1, t-test p value < 0...
March 31, 2017: Chemosphere
https://www.readbyqxmd.com/read/28403191/visceral-adipose-tissue-but-not-subcutaneous-adipose-tissue-is-associated-with-urine-and-serum-metabolites
#12
Inga Schlecht, Wolfram Gronwald, Gundula Behrens, Sebastian E Baumeister, Johannes Hertel, Jochen Hochrein, Helena U Zacharias, Beate Fischer, Peter J Oefner, Michael F Leitzmann
Obesity is a complex multifactorial phenotype that influences several metabolic pathways. Yet, few studies have examined the relations of different body fat compartments to urinary and serum metabolites. Anthropometric phenotypes (visceral adipose tissue (VAT), subcutaneous adipose tissue (SAT), the ratio between VAT and SAT (VSR), body mass index (BMI), waist circumference (WC)) and urinary and serum metabolite concentrations measured by nuclear magnetic resonance spectroscopy were measured in a population-based sample of 228 healthy adults...
2017: PloS One
https://www.readbyqxmd.com/read/28402322/nutritional-status-in-patients-with-phenylketonuria-using-glycomacropeptide-as-their-major-protein-source
#13
A Pinto, M F Almeida, P C Ramos, S Rocha, A Guimas, R Ribeiro, E Martins, A Bandeira, A MacDonald, J C Rocha
BACKGROUND/OBJECTIVES: Low phenylalanine (PHE), glycomacropeptide-based protein substitute (GMP) is an alternative to traditional L-amino acid supplements (AA) used in the dietary management of phenylketonuria (PKU). In a retrospective, longitudinal study, we report the nutritional status of PKU patients taking AA and GMP. SUBJECTS/METHODS: Eleven PKU patients aged 27±10 years (1 HPA, 4 mild and 6 classical PKU) on dietary treatment were evaluated (anthropometry, body composition, blood pressure measurements, biochemical markers including vitamin, mineral, lipids, carbohydrates and protein status/metabolism, and nutritional intake assessment) at two different annual reviews...
April 12, 2017: European Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28401628/metabolomics-reveals-new-metabolic-perturbations-in-children-with-type-1-diabetes
#14
Alfonso Galderisi, Paola Pirillo, Vittoria Moret, Matteo Stocchero, Antonina Gucciardi, Giorgio Perilongo, Carlo Moretti, Carlamaria Monciotti, Giuseppe Giordano, Eugenio Baraldi
OBJECTIVE: Using an untargeted metabolomics approach we investigated the metabolome of children with type 1 diabetes (T1D) in comparison with healthy peers and explored the contribution of HbA1c and clinical features to the observed difference. RESEARCH DESIGN AND METHODS: We enrolled children with T1D aged 6-15 years, attending the pediatric diabetes clinic of University of Padova (Italy). Healthy controls were enrolled on voluntary basis and matched for age, sex, pubertal status, body mass index (BMI)...
April 12, 2017: Pediatric Diabetes
https://www.readbyqxmd.com/read/28393748/gut-microbiota-of-mice-putatively-modifies-amino-acid-metabolism-in-the-host-brain
#15
Takahiro Kawase, Mao Nagasawa, Hiromi Ikeda, Shinobu Yasuo, Yasuhiro Koga, Mitsuhiro Furuse
Recently, it has been found that the gut microbiota influences functions of the host brain by affecting monoamine metabolism. The present study focused on the relationship between the gut microbiota and the brain amino acids. Specific pathogen-free (SPF) and germ-free (GF) mice were used as experimental models. Plasma and brain regions were sampled from mice at 7 and 16 weeks of age, and analysed for free d- and l-amino acids, which are believed to affect many physiological functions. At 7 weeks of age, plasma concentrations of d-aspartic acid (d-Asp), l-alanine (l-Ala), l-glutamine (l-Gln) and taurine were higher in SPF mice than in GF mice, but no differences were found at 16 weeks of age...
April 10, 2017: British Journal of Nutrition
https://www.readbyqxmd.com/read/28389705/metabolic-effects-of-insulin-in-a-human-model-of-ketoacidosis-combining-exposure-to-lipopolysaccharide-and-insulin-deficiency-a-randomised-controlled-crossover-study-in-individuals-with-type-1-diabetes
#16
Mads V Svart, Nikolaj Rittig, Ulla Kampmann, Thomas S Voss, Niels Møller, Niels Jessen
AIMS/HYPOTHESIS: Diabetic ketoacidosis (DKA) is often caused by concomitant systemic inflammation and lack of insulin. Here we used an experimental human model to test whether and how metabolic responses to insulin are impaired in the early phases of DKA with a specific focus on skeletal muscle metabolism. METHODS: Nine individuals with type 1 diabetes from a previously published cohort were investigated twice at Aarhus University Hospital using a 120 min infusion of insulin (3...
April 7, 2017: Diabetologia
https://www.readbyqxmd.com/read/28386663/a-child-with-phenylketonuria-and-focal-segmental-glomerulosclerosis-the-bright-side-of-proteinuria
#17
Fatma Rabah, Khalid Al-Thihli, Mohamed El-Naggari, Ibtisam B Elnour
Phenylketonuria (PKU) is the most common inborn error of amino acid metabolism. Phenylalanine hydroxylase is the underlying deficient enzyme. If left untreated, growth failure, microcephaly, global developmental delay, seizures and severe intellectual impairment would characterize the clinical picture of PKU. On the other side of protein homeostasis lies nephrotic syndrome. It is a well-known quantitative defect due to significant proteinuria. Focal segmental glomerulosclerosis (FSGS) is a special congenital variant affecting children and adults...
April 7, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28386239/skeletal-muscle-myomir-are-differentially-expressed-by-endurance-exercise-mode-and-combined-essential-amino-acid-and-carbohydrate-supplementation
#18
Lee M Margolis, Holly L McClung, Nancy E Murphy, Christopher T Carrigan, Stefan M Pasiakos
Skeletal muscle microRNAs (myomiR) expression is modulated by exercise, however, the influence of endurance exercise mode, combined with essential amino acid and carbohydrate (EAA+CHO) supplementation are not well defined. This study determined the effects of weighted versus non-weighted endurance exercise, with or without EAA+CHO ingestion on myomiR expression and their association with muscle protein synthesis (MPS). Twenty five adults performed 90 min of metabolically-matched (2.2 VO2 L·m(-1)) load carriage (LC; performed on a treadmill wearing a vest equal to 30% of individual body mass) or cycle ergometry (CE) exercise, during which EAA+CHO (10 g EAA and 46 g CHO) or non-nutritive control (CON) drinks were consumed...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28383617/plasma-amino-acids-of-the-transsulfuration-pathway-and-plasma-lactate-in-septic-patients
#19
C Chiarla, I Giovannini, G Miggiano
OBJECTIVES: In sepsis increasing plasma lactate, even if unrelated to hypoperfusion and hypoxia, is a cause of concern. Among the patterns associated with increasing lactate, several plasma amino acid (AA) abnormalities, more in particular those of sulfur AAs, have remained unexplored, and their assessment has been the purpose of our study. MATERIALS AND METHODS: A systematic and detailed analysis of 183 simultaneous determinations of plasma AA-grams and lactate, from 12 trauma surgery patients who had developed sepsis, was performed...
March 2017: La Clinica Terapeutica
https://www.readbyqxmd.com/read/28382600/treatment-adherence-and-psychological-wellbeing-in-maternal-carers-of-children-with-phenylketonuria-pku
#20
Emma Medford, Dougal Julian Hare, Katie Carpenter, Stewart Rust, Simon Jones, Anja Wittkowski
Phenylketonuria (PKU), a rare metabolic disorder, causes cognitive impairment unless treated with a strict, protein-restricted diet, but few studies have examined the relationship between treatment compliance and parental wellbeing. In the present study, 46 primary caregivers of children with PKU completed measures of psychological distress, parenting stress (related to caring for a child with an illness), resilience, perceived social support and child dependency. Treatment adherence was assessed using the proportion of blood phenylalanine concentrations within target range in the preceding year...
April 6, 2017: JIMD Reports
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