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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/28324191/planarian-homolog-of-puromycin-sensitive-aminopeptidase-djpsa-is-required-for-brain-regeneration
#2
Suge Wu, Bin Liu, Zuoqing Yuan, Xiufang Zhang, Hong Liu, Qiuxiang Pang, Bosheng Zhao
Puromycin-sensitive aminopeptidase (PSA) belongs to the M1 zinc metallopeptidase family. PSA is the most abundant aminopeptidase in the brain and plays a role in the metabolism of neuropeptides including those involved in neurodegeneration. A cDNA DjPsa was identified from the planarian Dugesia japonica cDNA library. It contains a 639-bp open reading frame corresponding to a deduced protein of 212 amino acids. Whole mount in situ hybridization revealed that DjPsa is expressed in the brain and ventral nerve cords of intact and regenerating animals and demonstrates a tissue and stage-specific expression pattern of DjPsa in developing embryos and larvae...
June 2017: Invertebrate Neuroscience: IN
https://www.readbyqxmd.com/read/28324172/enzymes-involved-in-branched-chain-amino-acid-metabolism-in-humans
#3
REVIEW
María M Adeva-Andany, Laura López-Maside, Cristóbal Donapetry-García, Carlos Fernández-Fernández, Cristina Sixto-Leal
Branched-chain amino acids (leucine, isoleucine and valine) are structurally related to branched-chain fatty acids. Leucine is 2-amino-4-methyl-pentanoic acid, isoleucine is 2-amino-3-methyl-pentanoic acid, and valine is 2-amino-3-methyl-butanoic acid. Similar to fatty acid oxidation, leucine and isoleucine produce acetyl-coA. Additionally, leucine generates acetoacetate and isoleucine yields propionyl-coA. Valine oxidation produces propionyl-coA, which is converted into methylmalonyl-coA and succinyl-coA. Branched-chain aminotransferase catalyzes the first reaction in the catabolic pathway of branched-chain amino acids, a reversible transamination that converts branched-chain amino acids into branched-chain ketoacids...
March 21, 2017: Amino Acids
https://www.readbyqxmd.com/read/28323986/chronic-intranasal-insulin-does-not-affect-hepatic-lipids-but-lowers-circulating-bcaas-in-healthy-male-subjects
#4
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/28318874/metabolic-profile-provides-prognostic-value-better-than-galectin-3-in-patients-with-heart-failure
#5
Chao-Hung Wang, Mei-Ling Cheng, Min-Hui Liu, Li-Tang Kuo, Ming-Shi Shiao
BACKGROUND: Metabolic profiles have been shown to provide prognostic information in patients with heart failure (HF). Galectin-3 (Gal-3), indicating cardiac fibrosis, is a documented biomarker of prognosis in HF. It is unknown whether metabolic profiles provide prognostic value better than Gal-3. METHODS AND RESULTS: This study analyzed 212 hospitalized HF patients, measuring metabolic score (composed by butyrylcarnitine, dimethylarginine/arginine ratio, spermidine, and total essential amino acids) and Gal-3...
March 16, 2017: Journal of Cardiology
https://www.readbyqxmd.com/read/28317865/complete-genome-sequencing-and-antibiotics-biosynthesis-pathways-analysis-of-streptomyces-lydicus-103
#6
Nan Jia, Ming-Zhu Ding, Hao Luo, Feng Gao, Ying-Jin Yuan
More and more new natural products have been found in Streptomyces species, which become the significant resource for antibiotics production. Among them, Streptomyces lydicus has been known as its ability of streptolydigin biosynthesis. Herein, we present the genome analysis of S. lydicus based on the complete genome sequencing. The circular chromosome of S. lydicus 103 comprises 8,201,357 base pairs with average GC content 72.22%. With the aid of KEGG analysis, we found that S. lydicus 103 can transfer propanoate to succinate, glutamine or glutamate to 2-oxoglutarate, CO2 and L-glutamate to ammonia, which are conducive to the the supply of amino acids...
March 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28316081/the-antidiabetic-drug-metformin-decreases-mitochondrial-respiration-and-tricarboxylic-acid-cycle-activity-in-cultured-primary-rat-astrocytes
#7
Michaela C Hohnholt, Eva-Maria Blumrich, Helle S Waagepetersen, Ralf Dringen
Metformin is an antidiabetic drug that is used daily by millions of patients worldwide. Metformin is able to cross the blood-brain barrier and has recently been shown to increase glucose consumption and lactate release in cultured astrocytes. However, potential effects of metformin on mitochondrial tricarboxylic acid (TCA) cycle metabolism in astrocytes are unknown. We investigated this by mapping (13) C labeling in TCA cycle intermediates and corresponding amino acids after incubation of primary rat astrocytes with [U-(13) C]glucose...
March 19, 2017: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/28315774/molecular-characterization-of-amp-activated-protein-kinase-%C3%AE-2-from-herbivorous-fish-megalobrama-amblycephala-and-responsiveness-to-glucose-loading-and-dietary-carbohydrate-levels
#8
Chao Xu, Wen-Bin Liu, Ding-Dong Zhang, Kai-Zhou Wang, Si-Lei Xia, Xiang-Fei Li
This study aimed to characterize the full-length cDNA of AMPKα2 in Megalobrama amblycephala, and evaluate its potential role in glucose homeostasis and carbohydrate metabolism. The cDNA obtained covered 1942bp with an open reading frame of 1635bp encoding 545 amino acids. Multiple alignments and phylogenetic analysis revealed a high homology (91-100%) among most fish and higher vertebrates. This AMPKα2 mRNA predominantly expressed in muscle, liver and brain, while little in gill and intestine. Then, the AMPKα2 expressions were determined in the muscle, liver and brain of fish subjected to a glucose load (injected intraperitoneally with 0, 1...
March 15, 2017: Comparative Biochemistry and Physiology. Part A, Molecular & Integrative Physiology
https://www.readbyqxmd.com/read/28315483/functional-proteomic-analysis-of-corticosteroid-pharmacodynamics-in-rat-liver-relationship-to-hepatic-stress-signaling-energy-regulation-and-drug-metabolism
#9
Vivaswath S Ayyar, Richard R Almon, Debra C DuBois, Siddharth Sukumaran, Jun Qu, William J Jusko
Corticosteroids (CS) are anti-inflammatory agents that cause extensive pharmacogenomic and proteomic changes in multiple tissues. An understanding of the proteome-wide effects of CS in liver and its relationships to altered hepatic and systemic physiology remains incomplete. Here, we report the application of a functional pharmacoproteomic approach to gain integrated insight into the complex nature of CS responses in liver in vivo. An in-depth functional analysis was performed using rich pharmacodynamic (temporal-based) proteomic data measured over 66h in rat liver following a single dose of methylprednisolone (MPL)...
March 14, 2017: Journal of Proteomics
https://www.readbyqxmd.com/read/28315295/proton-nmr-metabolic-profiling-of-csf-reveals-distinct-differentiation-of-meningitis-from-negative-controls
#10
Tanushri Chatterji, Suruchi Singh, Manodeep Sen, Ajai Kumar Singh, Gaurav Raj Agarwal, Deepak Kumar Singh, Janmejai Kumar Srivastava, Alka Singh, Rajendra Nath Srivastava, Raja Roy
BACKGROUND: Cerebrospinal fluid (CSF) is an essential bio-fluid of the central nervous system (CNS), playing a vital role in the protection of CNS and performing neuronal function regulation. The chemical composition of CSF varies during onset of meningitis, neurodegenerative disorders (positive controls) and in traumatic cases (negative controls). METHODS: The study design was broadly categorized into meningitis cases, negative controls and positive controls. Further differentiation among the three groups was carried out using Principal Component Analysis (PCA) followed by supervised Partial Least Square Discriminant Analysis (PLS-DA)...
March 14, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28314727/transcriptomic-analyses-elucidate-adaptive-differences-of-closely-related-strains-of-p-aeruginosa-in-fuel
#11
Thusitha S Gunasekera, Loryn L Bowen, Carol E Zhou, Susan C Howard-Byerly, William S Foley, Richard C Striebich, Larry C Dugan, Oscar N Ruiz
Pseudomonas aeruginosa can utilize hydrocarbons, but different strains have varying degrees of adaptation despite their highly conserved genome. P. aeruginosa ATCC 33988 is highly adapted to hydrocarbons while strain PAO1, a human pathogen, is less-adapted and degrades jet fuel at a slower rate than does ATCC 33988. We investigated fuel specific transcriptomic differences between these strains in order to ascertain the underling mechanisms utilized by the adapted strain to proliferate in fuel. During growth in fuel, the genes related to alkane degradation, heat-shock response, membrane proteins, efflux pumps and several novel genes were upregulated in ATCC 33988...
March 17, 2017: Applied and Environmental Microbiology
https://www.readbyqxmd.com/read/28314591/the-ubiquitin-binding-protein-tax1bp1-mediates-autophagasome-induction-and-the-metabolic-transition-of-activated-t-cells
#12
Michael I Whang, Rita M Tavares, Daniel I Benjamin, Michael G Kattah, Rommel Advincula, Daniel K Nomura, Jayanta Debnath, Barbara A Malynn, Averil Ma
During immune responses, naive T cells transition from small quiescent cells to rapidly cycling cells. We have found that T cells lacking TAX1BP1 exhibit delays in growth of cell size and cell cycling. TAX1BP1-deficient T cells exited G0 but stalled in S phase, due to both bioenergetic and biosynthetic defects. These defects were due to deficiencies in mTOR complex formation and activation. These mTOR defects in turn resulted from defective autophagy induction. TAX1BP1 binding of LC3 and GABARAP via its LC3-interacting region (LIR), but not its ubiquitin-binding domain, supported T cell proliferation...
March 21, 2017: Immunity
https://www.readbyqxmd.com/read/28314050/efficacy-of-rna-interference-knockdown-using-aerosolized-short-interfering-rnas-bound-to-nanoparticles-in-three-diverse-aphid-species
#13
M W Thairu, I H Skidmore, R Bansal, E Nováková, T E Hansen, H Li-Byarlay, S A Wickline, A K Hansen
RNA interference (RNAi) has emerged as a promising method for validating gene function; however, its utility in nonmodel insects has proven problematic, with delivery methods being one of the main obstacles. This study investigates a novel method of RNAi delivery in aphids, the aerosolization of short interfering RNA (siRNA)-nanoparticle complexes. By using nanoparticles as a siRNA carrier, the likelihood of cellular uptake is increased, when compared to methods previously used in insects. To determine the efficacy of this RNAi delivery system, siRNAs were aerosolized with and without nanoparticles in three aphid species: Acyrthosiphon pisum, Aphis glycines and Schizaphis graminum...
March 17, 2017: Insect Molecular Biology
https://www.readbyqxmd.com/read/28303408/methylene-blue-decreases-mitochondrial-lysine-acetylation-in-the-diabetic-heart
#14
Jessica M Berthiaume, Chia-Heng Hsiung, Alison B Austin, Sean P McBrayer, Mikayla M Depuydt, Margaret P Chandler, Masaru Miyagi, Mariana G Rosca
Diabetic cardiomyopathy is preceded by mitochondrial alterations, and progresses to heart failure. We studied whether treatment with methylene blue (MB), a compound that was reported to serve as an alternate electron carrier within the mitochondrial electron transport chain (ETC), improves mitochondrial metabolism and cardiac function in type 1 diabetes. MB was administered at 10 mg/kg/day to control and diabetic rats. Both echocardiography and hemodynamic studies were performed to assess cardiac function...
March 16, 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/28302921/deciphering-the-acute-cellular-phosphoproteome-response-to-irradiation-with-x-rays-protons-and-carbon-ions
#15
Martin Winter, Ivana Dokic, Julian Schlegel, Uwe Warnken, Jürgen Debus, Amir Abdollahi, Martina Schnölzer
Radiotherapy is a cornerstone of cancer therapy. The recently established particle therapy with raster-scanning protons and carbon ions landmarks a new era in the field of high-precision cancer medicine. However, molecular mechanisms governing radiation induced intracellular signaling remain elusive. Here, we present the first comprehensive proteomic and phosphoproteomic study applying stable isotope labeling by amino acids in cell culture (SILAC) in combination with high-resolution mass spectrometry to decipher cellular response to irradiation with X-rays, protons and carbon ions...
March 16, 2017: Molecular & Cellular Proteomics: MCP
https://www.readbyqxmd.com/read/28302759/preclinical-evaluation-of-11-c-sarcosine-as-a-substrate-of-proton-coupled-amino-acid-transporters-and-first-human-application-in-prostate-cancer
#16
Morand Piert, Xia Shao, David M Raffel, Mathew S Davenport, Jeffrey Montgomery, Lakshmi Kunju, Brian G Hockley, Javed Siddiqui, Peter J H Scott, Arul Chinnaiyan, Thekkelnaycke Rajandiran
Sarcosine is a known substrate of proton-coupled amino acid transporters (PAT), which are overexpressed in selected tissues and solid tumors. Sarcosine, an N-methyl derivative of the amino acid glycine and a metabolic product of choline, plays an important role for prostate cancer aggressiveness and progression. Methods: Carbon-11 radiolabeled sarcosine ((11)C-sarcosine) was tested as a new positron emission tomography (PET) imaging probe in comparison with (11)C-choline in two prostate cancer tumor xenograft models (DU-145 and PC-3)...
March 16, 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/28302506/xanthine-oxidase-inhibitory-activity-of-nicotino-isonicotinohydrazides-a-systematic-approach-from-in-vitro-in-silico-to-in-vivo-studies
#17
Humaira Zafar, Muhammad Hayat, Sumayya Saied, Momin Khan, Uzma Salar, Rizwana Malik, M Iqbal Choudhary, Khalid Mohammed Khan
Change in life style and eating habits has led to an increased prevalence of hyperuricemia worldwide. The role of hyperuricemia is no more restricted to gout, but it has a central role in progression of CVD, hypertension, metabolic syndrome, and arthritis. Among the different factors involved in regulation of serum uric acid, xanthine oxidase (XO) is the best pharmacological target to control the levels of serum uric acid as it catalyzes the final steps in uric acid production. In the current study, a systemic search for the inhibitors of xanthine oxidase, starting from synthesis to in vitro screening and leading to in vivo studies is presented...
February 28, 2017: Bioorganic & Medicinal Chemistry
https://www.readbyqxmd.com/read/28301735/glutaminolysis-a-hallmark-of-cancer-metabolism
#18
Lifeng Yang, Sriram Venneti, Deepak Nagrath
Glutamine is the most abundant circulating amino acid in blood and muscle and is critical for many fundamental cell functions in cancer cells, including synthesis of metabolites that maintain mitochondrial metabolism; generation of antioxidants to remove reactive oxygen species; synthesis of nonessential amino acids (NEAAs), purines and pyrimidines, and fatty acids for cellular replication; and activation of cell signaling. In light of the pleiotropic role of glutamine in cancer cells, a comprehensive understanding of glutamine metabolism is essential for the development of metabolic therapeutic strategies for targeting cancer cells...
March 8, 2017: Annual Review of Biomedical Engineering
https://www.readbyqxmd.com/read/28300770/transglycosylated-starch-improves-insulin-response-and-alters-lipid-and-amino-acid-metabolome-in-a-growing-pig-model
#19
Monica A Newman, Qendrim Zebeli, Eva Eberspächer, Dietmar Grüll, Timea Molnar, Barbara U Metzler-Zebeli
Due to the functional properties and physiological effects often associated with chemically modified starches, significant interest lies in their development for incorporation in processed foods. This study investigated the effect of transglycosylated cornstarch (TGS) on blood glucose, insulin, and serum metabolome in the pre- and postprandial phase in growing pigs. Eight jugular vein-catheterized barrows were fed two diets containing 72% purified starch (waxy cornstarch (CON) or TGS). A meal tolerance test (MTT) was performed with serial blood sampling for glucose, insulin, lipids, and metabolome profiling...
March 16, 2017: Nutrients
https://www.readbyqxmd.com/read/28300669/enhanced-plasma-protein-carbonylation-in-patients-with-myelodysplastic-syndromes
#20
Alžběta Hlaváčková, Jana Štikarová, Kristýna Pimková, Leona Chrastinová, Pavel Májek, Roman Kotlín, Jaroslav Čermák, Jiří Suttnar, Jan Evangelista Dyr
Myelodysplastic syndromes (MDS) represent a heterogeneous group of pre-leukemic disorders, characterized by ineffective hematopoiesis and the abnormal blood cell development of one or more lineages. Oxidative stress, as an important factor in the carcinogenesis of onco-hematological diseases, is also one of the known factors involved in the pathogenesis of MDS. An increase of reactive oxygen species (ROS) may lead to the oxidation of DNA, lipids, and proteins, thereby causing cell damage. Protein carbonylation caused by ROS is defined as an irreversible post-translational oxidative modification of amino acid side chains, and could play an important role in signaling processes...
March 12, 2017: Free Radical Biology & Medicine
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