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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/28320235/methanol-extract-of-nigella-sativa-seed-induces-changes-in-the-levels-of-neurotransmitter-amino-acids-in-male-rat-brain-regions
#2
Tarek El-Naggar, María Emilia Carretero, Carmen Arce, María Pilar Gómez-Serranillos
CONTEXT: Nigella sativa L. (Ranunculaceae) (NS) has been used for medicinal and culinary purposes. Different parts of the plant are used to treat many disorders. OBJECTIVE: This study investigates the effects of NS methanol extract on brain neurotransmitter amino acid levels. MATERIALS AND METHODS: We measured the changes in aspartate, glutamate, glycine and γ-aminobutyric acid in five brain regions of male Wistar rats after methanol extract treatment...
December 2017: Pharmaceutical Biology
https://www.readbyqxmd.com/read/28299915/metabolism-plays-the-key-roles-in-th-cells-differentiation
#3
A Hosseinzadeh, S Soukhtehzari, M Ghaedi, R Mansouri
The increasing rate of autoimmunity in recent decades cannot be related to only genetic instabilities and disorders. Diet can directly influence our health. Studies have shown that there is a relationship between nutritional elements and alteration in the immune system. Among immune cells, the function of T lymphocyte is important in directing immune response. T CD4+ cells lead other immune cells to respond to pathogens by secreting cytokines. HIV+ patients, who have largely lost their T CD4+ cells, are susceptible to opportunistic infections, which do not normally affect healthy people...
December 31, 2016: Reumatismo
https://www.readbyqxmd.com/read/28284832/prolonged-inhibition-of-inflammation-in-osteoarthritis-by-triamcinolone-acetonide-released-from-a-polyester-amide-microsphere-platform
#4
Imke Rudnik-Jansen, Sascha Colen, Julien Berard, Saskia Plomp, Ivo Que, Mattie van Rijen, Nina Woike, Annelies Egas, Gerjo van Osch, Erik van Maarseveen, Ken Messier, Alan Chan, Jens Thies, Laura Creemers
Controlled biomaterial-based corticosteroid release might circumvent multiple injections and the accompanying risks, such as hormone imbalance and muscle weakness, in osteoarthritic (OA) patients. For this purpose, microspheres were prepared from an amino acid-based polyester amide (PEA) platform and loaded with triamcinolone acetonide (TAA). TAA loaded microspheres were shown to release TAA for over 60days in PBS. Furthermore, the bioactivity lasted at least 28days, demonstrated by a 80-95% inhibition of PGE2 production using TNFα-stimulated chondrocyte culture, indicating inhibition of inflammation...
March 9, 2017: Journal of Controlled Release: Official Journal of the Controlled Release Society
https://www.readbyqxmd.com/read/28253350/sox10-contributes-to-the-balance-of-fate-choice-in-dorsal-root-ganglion-progenitors
#5
Mariana Delfino-Machín, Romain Madelaine, Giorgia Busolin, Masataka Nikaido, Sarah Colanesi, Karen Camargo-Sosa, Edward W P Law, Stefano Toppo, Patrick Blader, Natascia Tiso, Robert N Kelsh
The development of functional peripheral ganglia requires a balance of specification of both neuronal and glial components. In the developing dorsal root ganglia (DRGs), these components form from partially-restricted bipotent neuroglial precursors derived from the neural crest. Work in mouse and chick has identified several factors, including Delta/Notch signaling, required for specification of a balance of these components. We have previously shown in zebrafish that the Sry-related HMG domain transcription factor, Sox10, plays an unexpected, but crucial, role in sensory neuron fate specification in vivo...
2017: PloS One
https://www.readbyqxmd.com/read/28199774/sarcopenia-predicts-minimal-hepatic-encephalopathy-in-patients-with-liver-cirrhosis
#6
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/28182903/balancing-act-maintaining-amino-acid-levels-in-the-autistic-brain
#7
Thomas M Maynard, M Chiara Manzini
The ever-expanding number of genes that are mutated in autism is showing us how imbalances in fundamental cellular processes can lead to disease. A recent study by Tărlungeanu et al. (2016) identifies a form of ASD resulting from a failure of the brain to properly import amino acids.
February 8, 2017: Neuron
https://www.readbyqxmd.com/read/28178857/dietary-2-oxoglutarate-prevents-bone-loss-caused-by-neonatal-treatment-with-maximal-dexamethasone-dose
#8
Piotr Dobrowolski, Ewa Tomaszewska, Siemowit Muszyński, Tomasz Blicharski, Stefan G Pierzynowski
Synthetic glucocorticoids (GCs) are widely used in the variety of dosages for treatment of premature infants with chronic lung disease, respiratory distress syndrome, allergies, asthma, and other inflammatory and autoimmune conditions. Yet, adverse effects such as glucocorticoid-induced osteoporosis and growth retardation are recognized. Conversely, 2-oxoglutarate (2-Ox), a precursor of glutamine, glutamate, and collagen amino acids, exerts protective effects on bone development. Our aim was to elucidate the effect of dietary administered 2-Ox on bone loss caused by neonatal treatment with clinically relevant maximal therapeutic dexamethasone (Dex) dose...
January 1, 2017: Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28110268/metabolic-and-physiological-adjustment-of-suaeda-maritima-to-combined-salinity-and-hypoxia
#9
Jan H Behr, Alain Bouchereau, Solenne Berardocco, Charlotte E Seal, Timothy J Flowers, Christian Zörb
BACKGROUND AND AIMS: Suaeda maritima is a halophyte commonly found on coastal wetlands in the intertidal zone. Due to its habitat S. maritima has evolved tolerance to high salt concentrations and hypoxic conditions in the soil caused by periodic flooding. In the present work, the adaptive mechanisms of S. maritima to salinity combined with hypoxia were investigated on a physiological and metabolic level. METHODS: To compare the adaptive mechanisms to deficient, optimal and stressful salt concentrations, S...
January 20, 2017: Annals of Botany
https://www.readbyqxmd.com/read/28079404/free-amino-acids-in-fibromyalgia-syndrome-relationship-with-clinical-picture
#10
Valeria Ruggiero, Massimiliano Mura, Enrico Cacace, Benedetta Era, Marcella Peri, Giuseppina Sanna, Antonella Fais
The objectives of our study were to evaluate free amino acid (FAA) concentrations in the serum of patients affected by fibromyalgia syndrome (FMS) and to determine the relationships between FAA levels and FMS clinical parameters. Thus, serum amino acid concentrations were quantified (HPLC analysis) in 23 females with fibromyalgia (according to the American College of Rheumatology classification criteria) and 20 healthy females. The results showed significantly higher serum concentrations of aspartate, cysteine, glutamate, glycine, isoleucine, leucine, methionine, ornithine, phenylalanine, sarcosine, serine, taurine, tyrosine and valine in FMS patients vs...
April 2017: Scandinavian Journal of Clinical and Laboratory Investigation
https://www.readbyqxmd.com/read/28035535/the-role-of-the-gastrointestinal-microbiota-in-visceral-pain
#11
Kieran Rea, Siobhain M O'Mahony, Timothy G Dinan, John F Cryan
A growing body of preclinical and clinical evidence supports a relationship between the complexity and diversity of the microorganisms that inhabit our gut (human gastrointestinal microbiota) and health status. Under normal homeostatic conditions this microbial population helps maintain intestinal peristalsis, mucosal integrity, pH balance, immune priming and protection against invading pathogens. Furthermore, these microbes can influence centrally regulated emotional behaviour through mechanisms including microbially derived bioactive molecules (amino acid metabolites, short-chain fatty acids, neuropeptides and neurotransmitters), mucosal immune and enteroendocrine cell activation, as well as vagal nerve stimulation...
December 30, 2016: Handbook of Experimental Pharmacology
https://www.readbyqxmd.com/read/28029626/hemespred-structure-based-ligand-specific-heme-binding-residues-prediction-by-using-fast-adaptive-ensemble-learning-scheme
#12
Jian Zhang, Haiting Chai, Bo Gao, Guifu Yang, Zhiqiang Ma
Heme is an essential biomolecule that widely exists in numerous extant organisms. Accurately identifying heme binding residues (HEMEs) is of great importance in disease progression and drug development. In this study, a novel predictor named HEMEsPred was proposed for predicting HEMEs. First, several sequence- and structure-based features, including amino acid composition, motifs, surface preferences and secondary structure, were collected to construct feature matrices. Second, a novel fast-adaptive ensemble learning scheme was designed to overcome the serious class-imbalance problem as well as to enhance the prediction performance...
October 4, 2016: IEEE/ACM Transactions on Computational Biology and Bioinformatics
https://www.readbyqxmd.com/read/28012236/effects-of-lys-deficiency-and-lys-lys-dipeptide-on-cellular-apoptosis-and-amino-acids-metabolism
#13
Jie Yin, Yuying Li, Hui Han, Jie Zheng, Lijian Wang, Wenkai Ren, Shuai Chen, Fei Wu, Rejun Fang, Xingguo Huang, Chunyong Li, Bie Tan, Xia Xiong, Yuzhe Zhang, Gang Liu, Jiming Yao, Tiejun Li, Yulong Yin
SCOPE: Lysine (Lys) is a common limiting amino acids (AA) for humans and animals and plays an important role in cell proliferation and metabolism, while metabolism of Lys deficiency and its dipeptide is still obscure. Thus, this study mainly investigated the effects of Lys deficiency and Lys-Lys dipeptide on apoptosis and AA metabolism in vitro and in vivo models. METHODS AND RESULTS: Lys deficiency induced cell cycle arrest and apoptosis and upregulated Lys transporters in vitro and in vivo...
December 24, 2016: Molecular Nutrition & Food Research
https://www.readbyqxmd.com/read/27966614/imbalanced-positive-selection-maintains-the-functional-divergence-of-duplicated-dihydrokaempferol-4-reductase-genes
#14
Bing-Hong Huang, Yi-Wen Chen, Chia-Lung Huang, Jian Gao, Pei-Chun Liao
Gene duplication could be beneficial by functional division but might increase the risk of genetic load. The dynamics of duplicated paralogs number could involve recombination, positive selection, and functional divergence. Duplication of DIHYDROFLAVONOL 4-REDUCTASE (DFR) has been reported in several organisms and may have been retained by escape from adaptive conflict (EAC). In this study, we screened the angiosperm DFR gene focusing on a diversified genus Scutellaria to investigate how these duplicated genes are retained...
December 14, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27934656/the-safety-and-regulatory-process-for-amino-acids-in-europe-and-the-united-states
#15
REVIEW
Ashley Roberts
The safety of long-term, high-dose amino acid consumption marketed as dietary supplements or functional or medical foods requires regulatory clearance in the European Union through the novel food process or through the Dietary Supplement Health and Education Act or the GRAS (Generally Recognized As Safe) route in the United States. The safety assessment of high daily doses of amino acids for bodybuilding or other health benefits is expected to require human studies to support tolerability and safety. The need for human studies is based on the fact that there is little or no evidence of toxicity from the conduct of animal toxicity studies and because standard animal testing would be inappropriate because of the large dosages required to provide a suitable margin of safety when extrapolating from animals to humans...
December 2016: Journal of Nutrition
https://www.readbyqxmd.com/read/27905012/microbial-proteins-as-novel-industrial-biotechnology-hosts-to-treat-epilepsy
#16
REVIEW
Zareen Amtul, Amal A Aziz
Epilepsy is characterized by the hyperexcitability of various neuronal circuits that results due to the imbalance between glutamate-mediated excitation of voltage-gated cation channels and γ-amino butyric acid (GABA)-mediated inhibition of anion channels leading to aberrant, sporadic oscillations or fluctuations in neuronal electrical activity. Epilepsy with a risk of mortality and around 65 million sufferers of all ages all over the world is limited therapeutically with high rates of adverse reactions, lack of complete seizure control, and over 30% patients with refractory epilepsy...
December 1, 2016: Molecular Neurobiology
https://www.readbyqxmd.com/read/27779710/antihypertensive-therapy-improves-insulin-resistance-and-serum-levels-of-interleukin-6-and-10-in-spontaneously-hypertensive-rats-with-steatohepatitis
#17
Masaya Kozono, Hirofumi Uto, Rie Ibusuki, Shiho Arima, Kohei Oda, Hiroki Taguchi, Fumisato Sasaki, Yuichiro Nasu, Shinichi Hashimoto, Hitoshi Setoyama, Shuji Kanmura, Masatsugu Numata, Hirohito Tsubouchi, Akio Ido
Metabolic syndrome based on insulin resistance (IR) and hypertension is a risk factor for advanced liver disease and cardiovascular disease in patients with nonalcoholic steatohepatitis (NASH). The present study investigated the effects of severe hypertension induced by a high‑salt (HS) diet and antihypertensive therapy on the pathophysiological condition of spontaneously hypertensive rats (SHRs) with steatohepatitis. Steatohepatitis was induced using a choline-deficient, L-amino acid-defined diet (CDAA)...
December 2016: Molecular Medicine Reports
https://www.readbyqxmd.com/read/27775595/the-molecular-and-cellular-effect-of-homocysteine-metabolism-imbalance-on-human-health
#18
REVIEW
Henrieta Škovierová, Eva Vidomanová, Silvia Mahmood, Janka Sopková, Anna Drgová, Tatiana Červeňová, Erika Halašová, Ján Lehotský
Homocysteine (Hcy) is a sulfur-containing non-proteinogenic amino acid derived in methionine metabolism. The increased level of Hcy in plasma, hyperhomocysteinemia, is considered to be an independent risk factor for cardio and cerebrovascular diseases. However, it is still not clear if Hcy is a marker or a causative agent of diseases. More and more research data suggest that Hcy is an important indicator for overall health status. This review represents the current understanding of molecular mechanism of Hcy metabolism and its link to hyperhomocysteinemia-related pathologies in humans...
October 20, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27775533/hemespred-structure-based-ligand-specific-heme-binding-residues-prediction-by-using-fast-adaptive-ensemble-learning-scheme
#19
Jian Zhang, Haiting Chai, Bo Gao, Guifu Yang, Zhiqiang Ma
Heme is an essential biomolecule that widely exists in numerous extant organisms. Accurately identifying heme binding residues (HEMEs) is of great importance in disease progression and drug development. In this study, a novel predictor named HEMEsPred was proposed for predicting HEMEs. First, several sequence- and structure-based features, including amino acid composition, motifs, surface preferences and secondary structure, were collected to construct feature matrices. Second, a novel fast-adaptive ensemble learning scheme was designed to overcome the serious class-imbalance problem as well as to enhance the prediction performance...
October 4, 2016: IEEE/ACM Transactions on Computational Biology and Bioinformatics
https://www.readbyqxmd.com/read/27761676/multicompartment-analysis-of-protein-restricted-phenylketonuric-mice-reveals-amino-acid-imbalances-in-brain
#20
Kara R Vogel, Erland Arning, Teodoro Bottiglieri, K Michael Gibson
BACKGROUND: The mainstay of therapy for phenylketonuria (PKU) remains dietary protein restriction. Developmental and neurocognitive outcomes for patients, however, remain suboptimal. We tested the hypothesis that mice with PKU receiving protein-restricted diets would reveal disruptions of brain amino acids that shed light on these neurocognitive deficits. METHOD: Phenylalanine hydroxylase-deficient (PKU) mice and parallel controls (both wild-type and heterozygous) were fed custom diets containing 18, 6, and 4 % protein for 3 weeks, after which tissues (brain, liver, sera) were collected for amino acid analysis profiling...
October 19, 2016: Journal of Inherited Metabolic Disease
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