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Guanidinoacetic

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https://www.readbyqxmd.com/read/28808834/biochemical-and-behavioral-phenotype-of-agat-and-gamt-deficient-mice-following-long-term-creatine-monohydrate-supplementation
#1
Furhan Iqbal, Herald Hoeger, Gurt Lubec, Olaf Bodamer
The creatine/phosphocreatine system is essential for cellular phosphate coupled energy storage and production. We investigated the utility of creatine monohydrate supplementation in two different creatine deficient knockout mouse models. Following weaning, female Arginine: Glycine Amidinotransferase (AGAT) and Guanidinoacetate: methyltransferase (GAMT) knockouts and wild type mice were studied based on their genotypes and dietary supplementation (creatine free or 2% creatine monohydrate supplemented diet) for 10 weeks, using a series of behavioral tests and biochemical analyzes...
August 14, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28764030/experimental-and-computational-study-of-guanidinoacetic-acid-self-aggregation-in-aqueous-solution
#2
Milan Vraneš, Sergej Ostojić, Aleksandar Tot, Snežana Papović, Slobodan Gadžurić
In this work for the first time the physicochemical and thermal properties of guanidinoacetic acid (GAA) and its aqueous solutions have been performed to test for its viability as a potential dietary supplement. Thermal stability, viscosity, solubility and experimental density are determined. From measured densities the volumetric properties were estimated and discussed in the scope of GAA self-aggregation in aqueous solutions using experimental and computational results. Based on thermal stability and solubility measurements, it is found that GAA is more thermally stable but less soluble comparing to creatine due to a self-aggregation process that occurs at GAA concentrations higher than 0...
December 15, 2017: Food Chemistry
https://www.readbyqxmd.com/read/28758966/variability-of-creatine-metabolism-genes-in-children-with-autism-spectrum-disorder
#3
Jessie M Cameron, Valeriy Levandovskiy, Wendy Roberts, Evdokia Anagnostou, Stephen Scherer, Alvin Loh, Andreas Schulze
Creatine deficiency syndrome (CDS) comprises three separate enzyme deficiencies with overlapping clinical presentations: arginine:glycine amidinotransferase (GATM gene, glycine amidinotransferase), guanidinoacetate methyltransferase (GAMT gene), and creatine transporter deficiency (SLC6A8 gene, solute carrier family 6 member 8). CDS presents with developmental delays/regression, intellectual disability, speech and language impairment, autistic behaviour, epileptic seizures, treatment-refractory epilepsy, and extrapyramidal movement disorders; symptoms that are also evident in children with autism...
July 31, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28710553/metabolism-and-distribution-of-pharmacological-homoarginine-in-plasma-and-main-organs-of-the-anesthetized-rat
#4
Duygu Naile Günes, Arslan Arinc Kayacelebi, Erik Hanff, Joel Lundgren, Björn Redfors, Dimitrios Tsikas
L-Homoarginine (hArg) and guanidinoacetate (GAA) are produced from L-arginine (Arg) by the catalytic action of arginine:glycine amidinotransferase. Guanidinoacetate methyltransferase methylates GAA on its non-guanidine N atom to produce creatine. Arg and hArg are converted by nitric oxide synthase (NOS) to nitric oxide (NO). NO is oxidized to nitrite and nitrate which circulate in the blood and are excreted in the urine. Asymmetric dimethylarginine (ADMA), an NOS inhibitor, is widely accepted to be exclusively produced after asymmetric N (G)-methylation of Arg residues in proteins and their regular proteolysis...
July 14, 2017: Amino Acids
https://www.readbyqxmd.com/read/28705760/synthetic-analogues-of-cyanobacterial-alkaloid-cylindrospermopsin-and-their-toxicological-activity
#5
Christopher Cartmell, Daniel M Evans, Jessica M L Elwood, Hisham S Fituri, Patrick J Murphy, Thomas Caspari, Barbara Poniedziałek, Piotr Rzymski
Cylindrospermopsin (CYN) is a naturally occurring alkaloid produced by a variety of cyanobacteria and known to induce oxidative stress-mediated toxicity in eukaryotic cells. Despite extensive research on the mechanism of CYN toxicity, an understanding of the structural features responsible for this toxicity and the mechanism by which it can enter the cell are still not clear. It was established that the presence of both the uracil and guanidine groups is essential in biological activity of CYN whilst not much is known in this regard on the role of tether that separates them and the attached hydroxyl group...
July 10, 2017: Toxicology in Vitro: An International Journal Published in Association with BIBRA
https://www.readbyqxmd.com/read/28460226/co-administration-of-creatine-and-guanidinoacetic-acid-for-augmented-tissue-bioenergetics-a-novel-approach
#6
Sergej M Ostojic
A confined absorption of exogenous creatine through creatine transporter (CRT1) seems to hamper its optimal uptake in bioenergetical deficits. Co-administration of guanidinoacetic acid (GAA) along with creatine could target other transport channels besides CRT1, and supremely improve cellular levels of creatine. This innovative approach might tackle tissues difficult to reach with conventional creatine interventions, providing a potentially more effective and safe mixture in clinical pharmacology and therapeutics...
July 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28454702/creatine-biosynthesis-and-transport-by-the-term-human-placenta
#7
Stacey J Ellery, Paul A Della Gatta, Clinton R Bruce, Greg M Kowalski, Miranda Davies-Tuck, Joanne C Mockler, Padma Murthi, David W Walker, Rod J Snow, Hayley Dickinson
INTRODUCTION: Creatine is an amino acid derivative that is involved in preserving ATP homeostasis. Previous studies suggest an important role for the creatine kinase circuit for placental ATP turnover. Creatine is obtained from both the diet and endogenous synthesis, usually along the renal-hepatic axis. However, some tissues with a high-energy demand have an inherent capacity to synthesise creatine. In this study, we determined if the term human placenta has the enzymatic machinary to synthesise creatine...
April 2017: Placenta
https://www.readbyqxmd.com/read/28438604/first-reported-chinese-case-of-guanidinoacetate-methyltransferase-deficiency-in-a-4-year-old-child
#8
Weihua Sun, Yi Wang, Zhen Zu, Yi Jiang, Wei Lu, Huijun Wang, Bingbing Wu, Ping Zhang, Xiaomin Peng, Hao Zhou
Guanidinoacetate methyltransferase (GAMT) deficiency is a rare inherited disorder characterized by creatine (Cr) depletion and guanidinoacetate (GAA) accumulation in body fluids. We report the first identified Chinese case, diagnosed in a 4-year-old girl with onset of global developmental. Low Cr and high GAA levels were detected in her serum and urine, and low Cr level in her brain. Compound heterozygous variants in GAMT gene were found, including a previously reported variant at c.491dupG which was inherited from her mother and a novel variant at c...
July 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28403191/visceral-adipose-tissue-but-not-subcutaneous-adipose-tissue-is-associated-with-urine-and-serum-metabolites
#9
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/28379688/effects-of-doxorubicin-cisplatin-and-tamoxifen-on-the-metabolic-profile-of-human-breast-cancer-mcf-7-cells-as-determined-by-1-h-high-resolution-magic-angle-spinning-nuclear-magnetic-resonance
#10
Roberta M Maria, Wanessa F Altei, Heloisa S Selistre-de-Araujo, Luiz A Colnago
Doxorubicin (Doxo), cisplatin (Cis), and tamoxifen (Tamo) are part of many chemotherapeutic regimens. However, there have been limited studies of the way metabolism in breast cancer is affected by chemotherapy. We studied, through (1)H high-resolution magic angle spinning nuclear magnetic resonance (HR-MAS NMR) spectroscopy, the metabolic profile of human breast cancer MCF-7 control (Con) cells as well as MCF-7 cells treated with Tamo, Cis, and Doxo. (1)H HR-MAS NMR single-pulse spectra evidenced signals from the cell compounds, including fatty acids (membranes), water-soluble proteins, and metabolites...
April 25, 2017: Biochemistry
https://www.readbyqxmd.com/read/28341134/metabolic-changes-in-droplet-vitrified-semen-of-wild-endangered-persian-sturgeon-acipenser-persicus-borodin-1997
#11
Amirreza Abed-Elmdoust, Hamid Farahmand, Bagher Mojazi-Amiri, Gholamreza Rafiee, Ruhollah Rahimi
Comparative quantitative metabolite profiling can be used for better understanding of cell functions and dysfunctions in particular circumstances such as sperm banking which is an important approach for cryopreservation of endangered species. Cryopreservation techniques have some deleterious effects on spermatozoa which put the obtained results in controversy. Therefore, in the present study, quantitative (1)H NMR (Nuclear Magnetic Resonance) based metabolite profiling was conducted to evaluate metabolite changes related to energetics and some other detected metabolites in vitrified semen of critically endangered wild Acipenser persicus...
March 21, 2017: Cryobiology
https://www.readbyqxmd.com/read/28325734/does-maternal-fetal-transfer-of-creatine-occur-in-pregnant-sheep
#12
Syed Baharom, Robert De Matteo, Stacey Ellery, Paul Della Gatta, Clinton R Bruce, Greg M Kowalski, Nadia Hale, Hayley Dickinson, Richard Harding, David Walker, Rodney J Snow
Our aim was to determine the disposition of creatine in ovine pregnancy and whether creatine is transferred across the placenta from mother to fetus. Pregnant ewes received either 1) a continuous intravenous infusion of creatine monohydrate or saline from 122 to 131 days gestation, with maternal and fetal arterial blood and amniotic fluid samples collected daily for creatine analysis and fetal tissues collected at necropsy at 133 days for analysis of creatine content, or 2) a single systemic bolus injection of [(13)C]creatine monohydrate at 130 days of gestation, with maternal and fetal arterial blood, uterine vein blood, and amniotic fluid samples collected before and for 4 h after injection and analyzed for creatine, creatine isotopic enrichment, and guanidinoacetic acid (GAA; precursor of creatine) concentrations...
July 1, 2017: American Journal of Physiology. Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28285332/the-role-of-l-arginine-l-homoarginine-nitric-oxide-pathway-for-aortic-distensibility-and-intima-media-thickness-in-stroke-patients
#13
Arash Haghikia, Georgi Radoslavov Yanchev, Arslan Arinc Kayacelebi, Erik Hanff, Nils Bledau, Christian Widera, Kristina Sonnenschein, Aiden Haghikia, Karin Weissenborn, Johann Bauersachs, Udo Bavendiek, Dimitrios Tsikas
Asymmetric dimethylarginine (ADMA) and L-homoarginine (hArg) are L-arginine (Arg) metabolites derived from different pathways. Protein arginine N-methyltransferase (PRMT) and subsequent proteolysis of proteins containing methylarginine residues release ADMA. Arginine:glycine amidinotransferase (AGAT) converts Arg to hArg and guanidinoacetate (GAA). While high concentrations of ADMA and low concentrations of hArg in the blood have been established as cardiovascular risk markers, the cardiovascular relevance of GAA is still unexplored...
June 2017: Amino Acids
https://www.readbyqxmd.com/read/28220408/guanidinoacetate-methyltransferase-activity-in-lymphocytes-for-a-fast-diagnosis
#14
Lisette M Berends, Eduard A Struys, Birthe Roos, Ulbe Holwerda, Erwin E W Jansen, Gajja S Salomons, Mirjam M C Wamelink
INTRODUCTION: Guanidinoacetate methyltransferase (GAMT) deficiency is an inborn error of metabolism (IEM), clinically characterized by intellectual disability, developmental delay, seizures, and movement disorders. Biochemical diagnosis of GAMT deficiency is based on the measurement of creatine and guanidinoacetate in urine, plasma, or CSF and is confirmed genetically by DNA analysis or by enzyme assay in lymphoblasts or fibroblasts. To obtain enough cells, these cells need to be cultured for at least 1 month...
February 21, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28137860/creatine-maintains-intestinal-homeostasis-and-protects-against-colitis
#15
Emre Turer, William McAlpine, Kuan-Wen Wang, Tianshi Lu, Xiaohong Li, Miao Tang, Xiaoming Zhan, Tao Wang, Xiaowei Zhan, Chun-Hui Bu, Anne R Murray, Bruce Beutler
Creatine, a nitrogenous organic acid, replenishes cytoplasmic ATP at the expense of mitochondrial ATP via the phosphocreatine shuttle. Creatine levels are maintained by diet and endogenous synthesis from arginine and glycine. Glycine amidinotransferase (GATM) catalyzes the rate-limiting step of creatine biosynthesis: the transfer of an amidino group from arginine to glycine to form ornithine and guanidinoacetate. We screened 36,530 third-generation germline mutant mice derived from N-ethyl-N-nitrosourea-mutagenized grandsires for intestinal homeostasis abnormalities after oral administration of dextran sodium sulfate (DSS)...
February 14, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28119378/guanidinoacetate-methyltransferase-gamt-deficiency-a-rare-but-treatable-epilepsy
#16
William M Stern, Joel S Winston, Elaine Murphy, J Helen Cross, Josemir W Sander
Epilepsy commonly presents in childhood as part of a syndrome, and some such children may reach adult services without an underlying syndromic diagnosis. For adult neurologists taking over their care, it is often unclear how hard to search for an underlying diagnosis. The diagnostic yield may be small and such a diagnosis may not change management. Young adults with learning difficulties are also challenging to investigate, as they may not tolerate standard epilepsy tests.We present such a case in which simple tests identified a unifying diagnosis...
June 2017: Practical Neurology
https://www.readbyqxmd.com/read/28069926/creatine-enhances-mitochondrial-mediated-oligodendrocyte-survival-after-demyelinating-injury
#17
Kelly A Chamberlain, Kristen S Chapey, Sonia E Nanescu, Jeffrey K Huang
Chronic oligodendrocyte loss, which occurs in the demyelinating disorder multiple sclerosis (MS), contributes to axonal dysfunction and neurodegeneration. Current therapies are able to reduce MS severity, but do not prevent transition into the progressive phase of the disease, which is characterized by chronic neurodegeneration. Therefore, pharmacological compounds that promote oligodendrocyte survival could be beneficial for neuroprotection in MS. Here, we investigated the role of creatine, an organic acid involved in adenosine triphosphate (ATP) buffering, in oligodendrocyte function...
February 8, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28063512/tackling-guanidinoacetic-acid-for-advanced-cellular-bioenergetics
#18
REVIEW
Sergej M Ostojic
Tissues with high-energy output, such as the brain and skeletal muscle, suffer the most from impaired or depleted energy levels, with innovative nutritional approaches needed to effectively tackle metabolic deficits in bioenergetics. Here, we highlight the role of guanidinoacetic acid in the control and provision of cellular energy by its interaction with cellular transporters for taurine (SLC6 A6) and γ-aminobutyric acid (SLC6 A13), previously dismissed as "untargetable" carriers by other bioenergetics therapeutics...
February 2017: Nutrition
https://www.readbyqxmd.com/read/28052696/interactive-effects-of-temperature-and-dietary-supplementation-of-arginine-or-guanidinoacetic-acid-on-nutritional-and-physiological-responses-in-male-broiler-chickens
#19
COMPARATIVE STUDY
N Kodambashi Emami, A Golian, D D Rhoads, M Danesh Mesgaran
1. The aim of this experiment was to study the interactive effect of rearing temperature and dietary supplementation of arginine (Arg) or guanidinoacetic acid (GAA) on performance, gut morphology and ascites indices in broiler chickens raised under the same condition in the first 2 weeks and then reared under normal (23-26°C) or subnormal (17°C) ambient temperatures for the next 3 weeks. 2. This experiment was conducted as a split plot with 900 Ross 308 male broiler chicks that were allocated to two houses (as main plots); each consisted of 5 treatments (as sub-plots) with 6 replicates of 15 birds...
February 2017: British Poultry Science
https://www.readbyqxmd.com/read/28043349/effects-of-guanidinoacetic-acid-diet-supplementation-on-semen-quality-and-fertility-of-broiler-breeder-roosters
#20
Ramin Shahabi Tapeh, Mahdi Zhandi, Mojtaba Zaghari, Amir Akhlaghi
Decreased semen quality and fertility rate is a common feature in broiler breeder roosters. This decrease is associated with dysfunction of Sertoli cells and defective spermatogenesis. Guanidinoacetic acid (GAA), as a precursor of creatine, plays an important role in the proper functioning of Sertoli cells and energy metabolism in sperm. Twenty, 29-wk-old broiler breeder roosters (Ross 308) were randomly allotted to 4 treatment groups and fed diets supplemented with different levels of GAA, including 0 (GAA-0), 600 (GAA-600), 1200 (GAA-1200), and 1800 (GAA-1800) mg GAA/kg of diet for 26 successive weeks...
February 2017: Theriogenology
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