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Guanidinoacetate

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https://www.readbyqxmd.com/read/28438604/first-reported-chinese-case-of-guanidinoacetate-methyltransferase-deficiency-in-a-4-year-old-child
#1
Weihua Sun, Yi Wang, Zhen Zu, Jiang Yi, 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...
April 21, 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
#2
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
#3
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 12, 2017: Biochemistry
https://www.readbyqxmd.com/read/28341134/metabolic-changes-in-droplet-vitrified-semen-of-wild-endangered-persian-sturgeon-acipenser-persicus-borodin-1997
#4
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
#5
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: (i) 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; (ii) a single systemic bolus injection of (13)C labelled creatine monohydrate at 130 days of gestation, with maternal and fetal arterial blood, uterine vein blood and amniotic fluid samples collected prior to, and for 4 hours post injection and analysed for creatine, creatine isotopic enrichment, and guanidinoacetic acid (GAA, precursor of creatine) concentrations...
March 21, 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
#6
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...
March 11, 2017: Amino Acids
https://www.readbyqxmd.com/read/28220408/guanidinoacetate-methyltransferase-activity-in-lymphocytes-for-a-fast-diagnosis
#7
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
#8
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
#9
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...
January 24, 2017: Practical Neurology
https://www.readbyqxmd.com/read/28069926/creatine-enhances-mitochondrial-mediated-oligodendrocyte-survival-after-demyelinating-injury
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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