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Guanidinoacetate

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https://www.readbyqxmd.com/read/28069926/creatine-enhances-mitochondrial-mediated-oligodendrocyte-survival-following-demyelinating-injury
#1
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 ATP buffering, in oligodendrocyte function...
January 9, 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
#2
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
#3
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...
January 5, 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
#4
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
https://www.readbyqxmd.com/read/27859996/microphthalmia-associated-transcription-factor-ensures-the-elongation-of-axons-and-dendrites-in-the-mouse-frontal-cortex
#5
Koji Ohba, Kazuhisa Takeda, Tamio Furuse, Tomohiro Suzuki, Shigeharu Wakana, Takashi Suzuki, Hiroaki Yamamoto, Shigeki Shibahara
Long interspersed element-1 (LINE-1) is a mammalian transposable element, and its genomic insertion could cause neurological disorders in humans. Incidentally, LINE-1 is present in intron 3 of the microphthalmia-associated transcription factor (Mitf) gene of the black-eyed white mouse (Mitf(mi-bw) allele). Mice homozygous for the Mitf(mi-bw) allele show the white coat color with black eye and deafness. Here, we explored the functional consequences of the LINE-1 insertion in the Mitf gene using homozygous Mitf(mi-bw) mice on the C3H background (C3H-bw mice) or on the C57BL/6 background (bw mice)...
December 2016: Genes to Cells: Devoted to Molecular & Cellular Mechanisms
https://www.readbyqxmd.com/read/27784962/prolonged-feeding-with-guanidinoacetate-a-methyl-group-consumer-exacerbates-ethanol-induced-liver-injury
#6
Natalia A Osna, Dan Feng, Murali Ganesan, Priya F Maillacheruvu, David J Orlicky, Samuel W French, Dean J Tuma, Kusum K Kharbanda
AIM: To investigate the hypothesis that exposure to guanidinoacetate (GAA, a potent methyl-group consumer) either alone or combined with ethanol intake for a prolonged period of time would cause more advanced liver pathology thus identifying methylation defects as the initiator and stimulator for progressive liver damage. METHODS: Adult male Wistar rats were fed the control or ethanol Lieber DeCarli diet in the absence or presence of GAA supplementation. At the end of 6 wk of the feeding regimen, various biochemical and histological analyses were conducted...
October 14, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27701498/creatine-metabolism-detection-of-creatine-and-guanidinoacetate-in-saliva-of-healthy-subjects
#7
Lidia D Martínez, Miriam Bezard, Mabel Brunotto, Raquel Dodelson de Kremer
Creatine (Cr) plays an important role in storage and transmission of phosphate-bound energy. Cerebral creatine deficiency syndromes comprise three inherited defects in Cr biosynthesis and transport. The aim of this study was to investigate whether Cr and Guanidinoacetate (GAA) can be detected in saliva of healthy subjects and to establish the relationship between salivary and plasma levels of these molecules. An adapted gas chromatography (GC) method is described for the quantification of Cr and GAA biomarkers in saliva...
April 2016: Acta Odontológica Latinoamericana: AOL
https://www.readbyqxmd.com/read/27690568/a-single-session-of-exhaustive-exercise-markedly-decreases-circulating-levels-of-guanidinoacetic-acid-in-healthy-men-and-women
#8
Valdemar Stajer, Tatjana Trivic, Patrik Drid, Milan Vranes, Sergej M Ostojic
We evaluated the effects of exercise on circulating concentrations of guanidinoacetic acid (GAA) and creatine in 23 healthy volunteers subjected to running to exhaustion and free-weight bench-press to volitional failure. Blood was taken before and following each exercise session. Running induced a significant decrease in serum GAA by 20.1% (P < 0.001), while free-weight exercise reduced GAA by 11.7% (P < 0.001), suggesting the possible use of serum GAA as a novel biomarker of exhaustion.
July 19, 2016: Applied Physiology, Nutrition, and Metabolism, Physiologie Appliquée, Nutrition et Métabolisme
https://www.readbyqxmd.com/read/27650626/creatine-deficiency-syndrome-could-be-missed-easily-a-case-report-of-guanidinoacetate-methyltransferase-deficiency-presented-with-neurodevelopmental-delay-seizures-and-behavioral-changes-but-normal-structural-mri
#9
Iliyana Pacheva, Ivan Ivanov, Marin Penkov, Daliya Kancheva, Albena Jordanova, Mariya Ivanova
A case with GAMT deficiency (homozygous c.64dupG mutation) presented with neurodevelopmental delay, rare seizures, behavioral disturbances, and mild hypotonia, posing diagnostic challenges. Metabolic investigations showed low creatinine in plasma and urine (guanidinoacetate couldn't be investigated) and slightly elevated lactate. MRI was normal. Correct diagnosis was possible only after MR spectroscopy was performed at age 5½ years. A homozygous c.64dupG mutation of the GAMT gene was identified in the proband...
September 2016: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/27581622/creatine-supplementation-does-not-prevent-the-development-of-alcoholic-steatosis
#10
Murali Ganesan, Dan Feng, Ryan W Barton, Paul G Thomes, Benita L McVicker, Dean J Tuma, Natalia A Osna, Kusum K Kharbanda
BACKGROUND: Alcohol-induced reduction in the hepatocellular S-adenosylmethionine (SAM):S-adenosylhomocysteine (SAH) ratio impairs the activities of many SAM-dependent methyltransferases. These impairments ultimately lead to the generation of several hallmark features of alcoholic liver injury including steatosis. Guanidinoacetate methyltransferase (GAMT) is an important enzyme that catalyzes the final reaction in the creatine biosynthetic process. The liver is a major site for creatine synthesis which places a substantial methylation burden on this organ as GAMT-mediated reactions consume as much as 40% of all the SAM-derived methyl groups...
September 1, 2016: Alcoholism, Clinical and Experimental Research
https://www.readbyqxmd.com/read/27568578/quantitative-analysis-of-biomarkers-of-liver-and-kidney-injury-in-serum-and-urine-using-ultra-fast-liquid-chromatography-with-tandem-mass-spectrometry-coupled-with-a-hydrophilic-interaction-chromatography-column-application-to-monitor-injury-induced-by-euphorbiae
#11
Yuanyuan Zhang, Liqiang Gu, Yu Jiang, Kaishun Bi, Xiaohui Chen
Fast and sensitive monitoring of drug-induced liver and kidney injury in early stage is beneficial. An ultrafast liquid chromatography with tandem mass spectrometry assay was developed and validated to simultaneously determine ten endogenous biomarkers in serum and urine, including hippuric acid, phenylacetylglycine, 5-oxoproline, cholic acid, taurine, indoleacetic acid, 3-indoxyl sulfate, guanidinosuccinic acid, guanidinoacetic acid and uric acid. A CAPCELL CORE PC column (2.1 × 150 mm, 2.7 μm) was adopted for analytes separation...
October 2016: Journal of Separation Science
https://www.readbyqxmd.com/read/27560540/guanidinoacetic-acid-versus-creatine-for-improved-brain-and-muscle-creatine-levels-a-superiority-pilot-trial-in-healthy-men
#12
Sergej M Ostojic, Jelena Ostojic, Patrik Drid, Milan Vranes
In this randomized, double-blind, crossover trial, we evaluated whether 4-week supplementation with guanidinoacetic acid (GAA) is superior to creatine in facilitating creatine levels in healthy men (n = 5). GAA (3.0 g/day) resulted in a more powerful rise (up to 16.2%) in tissue creatine levels in vastus medialis muscle, middle-cerebellar peduncle, and paracentral grey matter, as compared with creatine (P < 0.05). These results indicate that GAA as a preferred alternative to creatine for improved bioenergetics in energy-demanding tissues...
September 2016: Applied Physiology, Nutrition, and Metabolism, Physiologie Appliquée, Nutrition et Métabolisme
https://www.readbyqxmd.com/read/27552474/potential-harmful-effects-of-dietary-supplements-in-sports-medicine
#13
Louise Deldicque, Marc Francaux
PURPOSE OF REVIEW: The purpose of this article is to collect the most recent data regarding the safety of well-known or emerging dietary supplements used by athletes. RECENT FINDINGS: From January 2014 to April 2016, about 30 articles have been published in the field. New data show that 90% of sports supplements contain trace of estrogenic endocrine disruptors, with 25% of them having a higher estrogenic activity than acceptable. About 50% of the supplements are contaminated by melamine, a source of nonprotein nitrogen...
November 2016: Current Opinion in Clinical Nutrition and Metabolic Care
https://www.readbyqxmd.com/read/27497517/dietary-guanidinoacetic-acid-increases-brain-creatine-levels-in-healthy-men
#14
Sergej M Ostojic, Jelena Ostojic, Patrik Drid, Milan Vranes, Pavle Jovanov
OBJECTIVE: Guanidinoacetic acid (GAA) is an experimental dietary additive that might act as a creatine source in tissues with high-energy requirements. In this case study, we evaluated brain levels of creatine in white matter, gray matter, cerebellum, and thalamus during 8 wk oral GAA administration in five healthy men and monitored the prevalence and severity of side effects of the intervention. METHODS: Volunteers were supplemented daily with 36 mg/kg body weight (BW) of GAA for the first 4 wk of the intervention; afterward GAA dosage was titrated ≤60 mg/kg BW of GAA daily...
January 2017: Nutrition
https://www.readbyqxmd.com/read/27477079/hypertensive-patients-exhibit-an-altered-metabolism-a-specific-metabolite-signature-in-urine-is-able-to-predict-albuminuria-progression
#15
Laura Gonzalez-Calero, Marta Martin-Lorenzo, Paula J Martínez, Montserrat Baldan-Martin, Gema Ruiz-Hurtado, Julian Segura, Fernando de la Cuesta, Maria G Barderas, Luis M Ruilope, Fernando Vivanco, Gloria Alvarez-Llamas
Hypertension (HTN) is increasing in prevalence, and albuminuria is a strong indicator of cardiovascular risk and renal damage progression. Despite blood pressure control with chronic treatment, a relevant subgroup of patients develop albuminuria. However, the biological factors responsible for albuminuria development and progression are underexplored. We aimed to identify key metabolic targets and biological pathways involved in the negative progression of cardiovascular and renal damage in hypertensives undergoing chronic treatment...
July 15, 2016: Translational Research: the Journal of Laboratory and Clinical Medicine
https://www.readbyqxmd.com/read/27408820/creatine-transporter-deficiency-novel-mutations-and-functional-studies
#16
O Ardon, M Procter, R Mao, N Longo, Y E Landau, A Shilon-Hadass, L V Gabis, C Hoffmann, M Tzadok, G Heimer, S Sada, B Ben-Zeev, Y Anikster
X-linked cerebral creatine deficiency (MIM 300036) is caused by deficiency of the creatine transporter encoded by the SLC6A8 gene. Here we report three patients with this condition from Israel. These unrelated patients were evaluated for global developmental delays and language apraxia. Borderline microcephaly was noted in one of them. Diagnosis was prompted by brain magnetic resonance imaging and spectroscopy which revealed normal white matter distribution, but absence of the creatine peak in all three patients...
September 2016: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/27359506/the-effects-of-guanidinoacetic-acid-supplementation-on-muscle-creatine-content-a-pilot-study-251-board-88-june-1-9-30-am-11-00-am
#17
Sergej M Ostojic, Patrik Drid, Jelena Ostojic, Jay R Hoffman
No abstract text is available yet for this article.
May 2016: Medicine and Science in Sports and Exercise
https://www.readbyqxmd.com/read/27233226/a-three-tier-algorithm-for-guanidinoacetate-methyltransferase-gamt-deficiency-newborn-screening
#18
Graham B Sinclair, Clara D M van Karnebeek, Manuel Ester, Frances Boyd, Tanya Nelson, Sylvia Stockler-Ipsiroglu, Hilary Vallance
BACKGROUND: Guanidinoacetate methyltransferase (GAMT) deficiency is a rare disorder of creatine biosynthesis presenting with epilepsy and developmental delay in infancy. Excellent developmental outcomes have been reported for infants treated from birth due to a family history. The BC Newborn Screening Program initiated a 3year pilot screening study for GAMT deficiency to evaluate the performance of a novel three-tiered screening approach. METHODS: Over 36months all bloodspots submitted for routine newborn screening were included in the pilot study (de-identified)...
July 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/27207152/methionine-metabolism-in-yucatan-miniature-swine
#19
Laura E McBreairty
Methionine is an essential amino acid which when not incorporated into protein, can be converted to S-adenosylmethionine, the universal methyl donor in over 200 transmethylation reactions, which include creatine and phosphatidylcholine (PC) synthesis, as well as deoxyribonucleic acid (DNA) methylation. Following transmethylation, homocysteine is formed, which can be converted to cysteine via transsulfuration or remethylated to methionine by receiving a methyl group from folate or betaine. Changes to methyl group availability in utero can lead to permanent changes in epigenetic patterns of DNA methylation, which has been implicated in "fetal programming", a phenomenon associated with poor nutrition during fetal development that results in low birth weight and disease in later life...
June 2016: Applied Physiology, Nutrition, and Metabolism, Physiologie Appliquée, Nutrition et Métabolisme
https://www.readbyqxmd.com/read/27038030/clinical-phenotype-biochemical-profile-and-treatment-in-19-patients-with-arginase-1-deficiency
#20
Martina Huemer, Daniel R Carvalho, Jaime M Brum, Özlem Ünal, Turgay Coskun, James D Weisfeld-Adams, Nina L Schrager, Sabine Scholl-Bürgi, Andrea Schlune, Markus G Donner, Martin Hersberger, Claudio Gemperle, Brunhilde Riesner, Hanno Ulmer, Johannes Häberle, Daniela Karall
BACKGROUND: Arginase 1 (ARG1) deficiency is a rare urea cycle disorder (UCD). This hypothesis-generating study explored clinical phenotypes, metabolic profiles, molecular genetics, and treatment approaches in a cohort of children and adults with ARG1 deficiency to add to our understanding of the underlying pathophysiology. METHODS: Clinical data were retrieved retrospectively from physicians using a questionnaire survey. Plasma aminoacids, guanidinoacetate (GAA), parameters indicating oxidative stress and nitric oxide (NO) synthesis as well as asymmetric dimethylarginine (ADMA) were measured at a single study site...
May 2016: Journal of Inherited Metabolic Disease
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