A methyltransferase that catalyzes the reaction of S-adenosyl-L-methionine and phenylethanolamine to yield S-adenosyl-L-homocysteine and N-methylphenylethanolamine. It can act on various phenylethanolamines and converts norepinephrine into epinephrine. (From Enzyme Nomenclature, 1992) EC 2.1.1.28.
An enzyme that catalyzes the METHYLATION of GLYCINE using S-ADENOSYLMETHIONINE to form SARCOSINE with the concomitant production of S-ADENOSYLHOMOCYSTEINE.
The active sympathomimetic hormone from the ADRENAL MEDULLA. It stimulates both the alpha- and beta- adrenergic systems, causes systemic VASOCONSTRICTION and gastrointestinal relaxation, stimulates the HEART, and dilates BRONCHI and cerebral vessels. It is used in ASTHMA and CARDIAC FAILURE and to delay absorption of local ANESTHETICS.
The inner portion of the adrenal gland. Derived from ECTODERM, adrenal medulla consists mainly of CHROMAFFIN CELLS that produces and stores a number of NEUROTRANSMITTERS, mainly adrenaline (EPINEPHRINE) and NOREPINEPHRINE. The activity of the adrenal medulla is regulated by the SYMPATHETIC NERVOUS SYSTEM.
A norepinephrine derivative used as a vasoconstrictor agent.
A group of ISOQUINOLINES in which the nitrogen containing ring is protonated. They derive from the non-enzymatic Pictet-Spengler condensation of CATECHOLAMINES with ALDEHYDES.
Nonparasitic free-living flatworms of the class Turbellaria. The most common genera are Dugesia, formerly Planaria, which lives in water, and Bipalium, which lives on land. Geoplana occurs in South America and California.
Relatively undifferentiated cells that retain the ability to divide and proliferate throughout postnatal life to provide progenitor cells that can differentiate into specialized cells.
The external, nonvascular layer of the skin. It is made up, from within outward, of five layers of EPITHELIUM: (1) basal layer (stratum basale epidermidis); (2) spinous layer (stratum spinosum epidermidis); (3) granular layer (stratum granulosum epidermidis); (4) clear layer (stratum lucidum epidermidis); and (5) horny layer (stratum corneum epidermidis).
Progressive restriction of the developmental potential and increasing specialization of function that leads to the formation of specialized cells, tissues, and organs.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
A class of free-living freshwater flatworms of North America.
The physiological renewal, repair, or replacement of tissue.
High molecular weight polymers containing a mixture of purine and pyrimidine nucleotides chained together by ribose or deoxyribose linkages.
Enzymes that catalyze the template-directed incorporation of ribonucleotides into an RNA chain. EC 2.7.7.-.
A large collection of DNA fragments cloned (CLONING, MOLECULAR) from a given organism, tissue, organ, or cell type. It may contain complete genomic sequences (GENOMIC LIBRARY) or complementary DNA sequences, the latter being formed from messenger RNA and lacking intron sequences.
Alkaloid isolated from seeds of Peganum harmala L., Zygophyllaceae. It is identical to banisterine, or telepathine, from Banisteria caapi and is one of the active ingredients of hallucinogenic drinks made in the western Amazon region from related plants. It has no therapeutic use, but (as banisterine) was hailed as a cure for postencephalitic Parkinson disease in the 1920's.
Polymers made up of a few (2-20) nucleotides. In molecular genetics, they refer to a short sequence synthesized to match a region where a mutation is known to occur, and then used as a probe (OLIGONUCLEOTIDE PROBES). (Dorland, 28th ed)
Enzyme that catalyzes the movement of a methyl group from S-adenosylmethionone to a catechol or a catecholamine.
An enzyme that catalyzes the oxidative deamination of naturally occurring monoamines. It is a flavin-containing enzyme that is localized in mitochondrial membranes, whether in nerve terminals, the liver, or other organs. Monoamine oxidase is important in regulating the metabolic degradation of catecholamines and serotonin in neural or target tissues. Hepatic monoamine oxidase has a crucial defensive role in inactivating circulating monoamines or those, such as tyramine, that originate in the gut and are absorbed into the portal circulation. (From Goodman and Gilman's, The Pharmacological Basis of Therapeutics, 8th ed, p415) EC 1.4.3.4.
Precursor of epinephrine that is secreted by the adrenal medulla and is a widespread central and autonomic neurotransmitter. Norepinephrine is the principal transmitter of most postganglionic sympathetic fibers and of the diffuse projection system in the brain arising from the locus ceruleus. It is also found in plants and is used pharmacologically as a sympathomimetic.
Databases devoted to knowledge about specific genes and gene products.
Transferases are enzymes transferring a group, for example, the methyl group or a glycosyl group, from one compound (generally regarded as donor) to another compound (generally regarded as acceptor). The classification is based on the scheme "donor:acceptor group transferase". (Enzyme Nomenclature, 1992) EC 2.
A transferase that catalyzes the addition of aliphatic, aromatic, or heterocyclic FREE RADICALS as well as EPOXIDES and arene oxides to GLUTATHIONE. Addition takes place at the SULFUR. It also catalyzes the reduction of polyol nitrate by glutathione to polyol and nitrite.
A urea cycle enzyme that catalyzes the formation of orthophosphate and L-citrulline (CITRULLINE) from CARBAMOYL PHOSPHATE and L-ornithine (ORNITHINE). Deficiency of this enzyme may be transmitted as an X-linked trait. EC 2.1.3.3.
An inherited urea cycle disorder associated with deficiency of the enzyme ORNITHINE CARBAMOYLTRANSFERASE, transmitted as an X-linked trait and featuring elevations of amino acids and ammonia in the serum. Clinical features, which are more prominent in males, include seizures, behavioral alterations, episodic vomiting, lethargy, and coma. (Menkes, Textbook of Child Neurology, 5th ed, pp49-50)
An amino acid produced in the urea cycle by the splitting off of urea from arginine.
A pyridoxal-phosphate protein, believed to be the rate-limiting compound in the biosynthesis of polyamines. It catalyzes the decarboxylation of ornithine to form putrescine, which is then linked to a propylamine moiety of decarboxylated S-adenosylmethionine to form spermidine.
An enzyme that catalyzes the conversion of carbamoyl phosphate and L-aspartate to yield orthophosphate and N-carbamoyl-L-aspartate. (From Enzyme Nomenclature, 1992) EC 2.1.3.2.
The monoanhydride of carbamic acid with PHOSPHORIC ACID. It is an important intermediate metabolite and is synthesized enzymatically by CARBAMYL-PHOSPHATE SYNTHASE (AMMONIA) and CARBAMOYL-PHOSPHATE SYNTHASE (GLUTAMINE-HYDROLYZING).
A group of enzymes that catalyze the transfer of carboxyl- or carbamoyl- groups. EC 2.1.3.
A disorder with chronic or recurrent colonic symptoms without a clearcut etiology. This condition is characterized by chronic or recurrent ABDOMINAL PAIN, bloating, MUCUS in FECES, and an erratic disturbance of DEFECATION.
A malabsorption syndrome resulting from extensive operative resection of the SMALL INTESTINE, the absorptive region of the GASTROINTESTINAL TRACT.
Chronic or recurrent colonic disorders without an identifiable structural or biochemical explanation. The widely recognized IRRITABLE BOWEL SYNDROME falls into this category.
A group of symptoms that are two- to three-fold more common in those who work in large, energy-efficient buildings, associated with an increased frequency of headaches, lethargy, and dry skin. Clinical manifestations include hypersensitivity pneumonitis (ALVEOLITIS, EXTRINSIC ALLERGIC); allergic rhinitis (RHINITIS, ALLERGIC, PERENNIAL); ASTHMA; infections, skin eruptions, and mucous membrane irritation syndromes. Current usage tends to be less restrictive with regard to the type of building and delineation of complaints. (From Segen, Dictionary of Modern Medicine, 1992)
A characteristic symptom complex.
An acute, diffuse, and suppurative inflammation of loose connective tissue, particularly the deep subcutaneous tissues, and sometimes muscle, which is most commonly seen as a result of infection of a wound, ulcer, or other skin lesions.

Isolation and characterization of the gene coding for the amidinotransferase involved in the biosynthesis of phaseolotoxin in Pseudomonas syringae pv. phaseolicola. (1/58)

Pseudomonas syringae pv. phaseolicola is the causal agent of the "halo blight" disease of beans. A key component in the development of the disease is a nonhost-specific toxin, Ndelta-(N'-sulphodiaminophosphinyl)-ornithyl-alanyl-homoarginine, known as phaseolotoxin. The homoarginine residue in this molecule has been suggested to be the product of L-arginine:lysine amidinotransferase activity, previously detected in extracts of P. syringae pv. phaseolicola grown under conditions of phaseolotoxin production. We report the isolation and characterization of an amidinotransferase gene (amtA) from P. syringae pv. phaseolicola coding for a polypeptide of 362 residues (41.36 kDa) and showing approximately 40% sequence similarity to L-arginine:inosamine-phosphate amidinotransferase from three species of Streptomyces spp. and 50.4% with an L-arginine:glycine amidinotransferase from human mitochondria. The cysteine, histidine, and aspartic acid residues involved in substrate binding are conserved. Furthermore, expression of the amtA and argK genes and phaseolotoxin production occurs at 18 degrees C but not at 28 degrees C. An amidinotransferase insertion mutant was obtained that lost the capacity to synthesize homoarginine and phaseolotoxin. These results show that the amtA gene isolated is responsible for the amidinotransferase activity detected previously and that phaseolotoxin production depends upon the activity of this gene.  (+info)

Arginine:glycine amidinotransferase deficiency: the third inborn error of creatine metabolism in humans. (2/58)

Arginine:glycine amidinotransferase (AGAT) catalyzes the first step of creatine synthesis, resulting in the formation of guanidinoacetate, which is a substrate for creatine formation. In two female siblings with mental retardation who had brain creatine deficiency that was reversible by means of oral creatine supplementation and had low urinary guanidinoacetate concentrations, AGAT deficiency was identified as a new genetic defect in creatine metabolism. A homozygous G-A transition at nucleotide position 9297, converting a tryptophan codon (TGG) to a stop codon (TAG) at residue 149 (T149X), resulted in undetectable cDNA, as investigated by reverse-transcription PCR, as well as in undetectable AGAT activity, as investigated radiochemically in cultivated skin fibroblasts and in virus-transformed lymphoblasts of the patients. The parents were heterozygous for the mutant allele, with intermediate residual AGAT activities. Recognition and treatment with oral creatine supplements may prevent neurological sequelae in affected patients.  (+info)

Methylation demand and homocysteine metabolism: effects of dietary provision of creatine and guanidinoacetate. (3/58)

S-adenosylmethionine, formed by the adenylation of methionine via S-adenosylmethionine synthase, is the methyl donor in virtually all known biological methylations. These methylation reactions produce a methylated substrate and S-adenosylhomocysteine, which is subsequently metabolized to homocysteine. The methylation of guanidinoacetate to form creatine consumes more methyl groups than all other methylation reactions combined. Therefore, we examined the effects of increased or decreased methyl demand by these physiological substrates on plasma homocysteine by feeding rats guanidinoacetate- or creatine-supplemented diets for 2 wk. Plasma homocysteine was significantly increased (~50%) in rats maintained on guanidinoacetate-supplemented diets, whereas rats maintained on creatine-supplemented diets exhibited a significantly lower (~25%) plasma homocysteine level. Plasma creatine and muscle total creatine were significantly increased in rats fed the creatine-supplemented or guanidinoacetate-supplemented diets. The activity of kidney L-arginine:glycine amidinotransferase, the enzyme catalyzing the synthesis of guanidinoacetate, was significantly decreased in both supplementation groups. To examine the role of the liver in mediating these changes in plasma homocysteine, isolated rat hepatocytes were incubated with methionine in the presence and absence of guanidinoacetate and creatine, and homocysteine export was measured. Homocysteine export was significantly increased in the presence of guanidinoacetate. Creatine, however, was without effect. These results suggest that homocysteine metabolism is sensitive to methylation demand imposed by physiological substrates.  (+info)

On the control of arginine metabolism in chicken kidney and liver. (4/58)

Arginases have been found to be located on the external side of the inner mitochondrial membrane of chicken kidney and liver. Transamidinase has been detected within the liver mitochondrial matrix space. Arginases and transamidinase act upon two different intracellular arginine pools. Penetration of arginine into the matrix space occurs only in respring mitochondria and in the presence of anions such as acetate and phosphate; D-arginine, L-ornithine, D-'ornithine and L-lysine penetrate with the same modalities. L-Histidine penetrates only kidney mitochondria. Because of transamidinase compartmentation, the rate of creatine synthesis is influenced by the rate of penetration of arginine into the mitochondria.  (+info)

Guanidinoacetate and creatine plus creatinine assessment in physiologic fluids: an effective diagnostic tool for the biochemical diagnosis of arginine:glycine amidinotransferase and guanidinoacetate methyltransferase deficiencies. (5/58)

BACKGROUND: Disorders of creatine metabolism arise from genetic alterations of arginine:glycine amidinotransferase (AGAT), guanidinoacetate methyltransferase (GAMT), and the creatine transporter. We developed a strategy for the detection of AGAT and GAMT defects by measurement of guanidinoacetate (GAA) and creatine plus creatinine (Cr+Crn) in biological fluids. METHODS: Three patients with AGAT deficiency from the same pedigree and their eight relatives, as well as a patient affected by a GAMT defect and his parents were analyzed by a new HPLC procedure in comparison with 90 controls. The method, which uses precolumn derivatization with benzoin, separation with a reversed-phase column, and fluorescence detection, has shown good precision and sensitivity and requires minimal sample handling. RESULTS: In the three AGAT patients, plasma GAA was 0.01-0.04 micro mol/L [mean (SD) for neurologically normal controls was 1.16 (0.59) micromol/L], Cr+Crn was 15-29 micro mol/L [reference limit in our laboratory, 79 (38) micromol/L]. Urinary GAA was 2.4-5.8 micro mol/L [reference, 311 (191) micromol/L], and Cr+Crn was 2.1-3.3 mmol/L [reference, 9.9 (4.1) mmol/L]. We found a smaller decrease in GAA and Cr+Crn in some carriers of an AGAT defect. In the patient with GAMT deficiency, plasma and urine GAA was increased (18.6 and 1783 micromol/L, respectively), and Cr+Crn was decreased in plasma (10.7 micromol/L) and urine (2.1 mmol/L). GAA was increased in the parents' plasmas and in the mother's urine. CONCLUSION: The assessment of GAA is a new tool for the detection of both GAMT and AGAT deficiencies.  (+info)

Fresh and cultured thyroid gland: survival and function after implantation. (6/58)

Isogeneic or allogeneic thyroid glands were implanted into thyroidectomized recipeint rats. These grafts, either fresh or cultured, were placed in hamstring muscle pocket or under the renal capsule. Survival and function of the grafts were evaluated by: restoration of normal levels of serum thyroxine, weight gain, kidney transamidinase (a thyroxine-induced enzyme), and histological appearance of re-excised implants. A isografts, fresh and cultured, functioned well as ectopic thyroid glands, though restoration of normal serum thyroxine levels was more rapid for the fresh implants. Fresh allografts functioned transiently but then failed due to rejection. No function was detected for cultured allografts, and rejection was seen histologically. The rat thyroid allograft therefore differs from the rat parathyroid allograft, which often can function for several months despite histological evidence of rejection. Maintenance in tissue culture prior to implantation does not appear to alter the longterm immunogenicity of either organ.  (+info)

Gatm, a creatine synthesis enzyme, is imprinted in mouse placenta. (7/58)

To increase our understanding of imprinting and epigenetic gene regulation, we undertook a search for new imprinted genes. We identified Gatm, a gene that encodes l-arginine:glycine amidinotransferase, which catalyzes the rate-limiting step in the synthesis of creatine. In mouse, Gatm is expressed during development and is imprinted in the placenta and yolk sac, but not in embryonic tissues. The Gatm gene maps to mouse chromosome 2 in a region not previously shown to contain imprinted genes. To determine whether Gatm is located in a cluster of imprinted genes, we investigated the expression pattern of genes located near Gatm: Duox1-2, Slc28a2, Slc30a4 and a transcript corresponding to LOC214616. We found no evidence that any of these genes is imprinted in placenta. We show that a CpG island associated with Gatm is unmethylated, as is a large CpG island associated with a neighboring gene. This genomic screen for novel imprinted genes has elucidated a new connection between imprinting and creatine metabolism during embryonic development in mammals.  (+info)

Studies on the catabolism of Ng-methylarginine, Ng, Ng-dimethylarginine and Ng, Ng-dimethylarginine in the rabbit. (8/58)

1. The routes of elimination of Ng-methylarginine, Ng, Ng-dimethylarginine and Ng, Ng-dimethylarginine were investigated in the rabbit. 2. Analyses showed low plasma concentrations of these amino acids (around 1 nmol/ml) and ratios similar to those found in tissue proteins. The concentrations of these amino acids in extracts of brain, kidney, liver and spleen were similar except that liver had a lower concentration of Ng-methylarginine and Ng, Ng-dimethylarginine. Cerebrospinal fluid contained traces of each amino acid.  (+info)

At least two mutations in the GATM gene cause arginine:glycine amidinotransferase deficiency, a disorder that involves delayed development, intellectual disability, and in some cases muscle weakness. These mutations result in the production of an abnormally shortened arginine:glycine amidinotransferase enzyme or disrupt how genetic information is pieced together to make a blueprint for producing the enzyme.. GATM gene mutations interfere with the ability of the arginine:glycine amidinotransferase enzyme to participate in creatine synthesis, resulting in a shortage of creatine. The effects of arginine:glycine amidinotransferase deficiency are most severe in organs and tissues that require large amounts of energy, especially the brain. ...
Glycine amidinotransferase definition at Dictionary.com, a free online dictionary with pronunciation, synonyms and translation. Look it up now!
1. Walker JB. Creatine: biosynthesis, regulation, function. Adv Enzymol Relat Areas Mol Biol. 1979;50:177-242 2. Wyss M, Kaddurah-Daouk R. Creatine and creatinine metabolism. Physiol Rev. 2000;80:1107-213 3. Van Pilsum JF. Evidence for a dual role of creatine in the regulation of kidney transamidinase activities in the rat. J Nutr. 1971;101:1085-92 4. McGuire DM, Grioss MD, Van Pilsum JF. et al. Repression of rat kidney L-arginine:glycine amidinotransferase synthesis by creatine at a pretranslational level. J Biol Chem. 1984;259:12034-8 5. Edison EE, Brosnan ME, Meyer C. et al. Creatine synthesis: production of guanidinoacetate by the rat and human kidney in vivo. Am J Physiol Renal Physiol. 2007;293:F1799-804 6. Snow RJ, Murphy RM. Creatine and the creatine transporter: a review. Mol Cell Biochem. 2001;224:169-81 7. Brosnan JT, Brosnan ME. Creatine: endogenous metabolite, dietary, and therapeutic supplement. Annu Rev Nutr. 2007;27:241-61 8. Stead LM, Au KP, Jacobs RL. et al. Methylation demand ...
Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development (By similarity).
Gatm antibody (glycine amidinotransferase (L-arginine:glycine amidinotransferase)) for WB. Anti-Gatm pAb (GTX124323) is tested in Zebrafish samples. 100% Ab-Assurance.
As a member of the wwPDB, the RCSB PDB curates and annotates PDB data according to agreed upon standards. The RCSB PDB also provides a variety of tools and resources. Users can perform simple and advanced searches based on annotations relating to sequence, structure and function. These molecules are visualized, downloaded, and analyzed by users who range from students to specialized scientists.
1jdw: Function and biology annotation of CRYSTAL STRUCTURE AND MECHANISM OF L-ARGININE: GLYCINE AMIDINOTRANSFERASE: A MITOCHONDRIAL ENZYME INVOLVED IN CREATINE BIOSYNTHESIS. Includes SCOP, CATH, InterPro, GO and Intenz annotation.
Creatine synthesis is required in adu lt anima ls to rep lace creatine that is spontaneous ly converted to creatinine and excreted in the urine. Additiona l ly, in growing anima ls it is necessary to
Creatine in the form of creatine phosphate plays an important role in ATP regeneration (Fig.1). The human body excretes about two grams of creatine in the form of creatinine via urine daily. Humans replenish creatine stores via either diet or de novo synthesis from glycine and arginine. Creatine is mainly found in red meat (muscle); it is scarce in other types of meat and is largely absent in plants. Vegans and vegetarians rely on de novo synthesis of creatine and place high demand for glycine, arginine, and SAM (methyl donor for GAMT enzyme, the key enzyme in creatine synthesis). It is estimated that 75% of SAH produced in the human body is from GAMT activity. Studies showed that vegans and vegetarians tend to have higher homocysteine levels than the general population and hence elevated SAH.. Glycine is the amino acid that is most frequently conjugated to toxins in human. Glycine is also in high demand for creatine synthesis in vegetarians, vegans, and omnivores with low intake of red meat ...
As a neurotransmitter, glycine affects the functioning of the nervous system, so it is responsible for suppressing the signs of depression, is beneficial for concentration and memory, and is used at bedtime to improve its quality and facilitate the building and regeneration of muscles. In addition, it participates in the synthesis of erythrocytes, haemoglobin and nucleic acid components, is present in the bodys detoxifying processes, participates in glucose and creatine biosynthesis and acts anti-sour and anti-inflammatory.. Glycine is a substance that does not occur on its own. It is, however, a component of numerous supplements - often found in testosterone boosters, creatine stacks, amino acid or carbohydrate protein. According to research, glycine sales last year increased fivefold. It is assumed that supplementation should be 1-3 g of product containing it in its composition. Glycine is also found in natural foods, both in animal and vegetable products, so varied diets. It will also be a ...
(1982) Thomasset et al. FEBS Letters. Cellular ornithine biosynthesis could be expected to play a significant role in putrescine formation and hence in growth. Two enzymes are involved in ornithine biosynthesis: arginase and transamidinase. These enzyme activities were studied in two human melano...
Supports protein and creatine synthesis, nitric oxide production Helps the detoxification of ammonia in the body Conditionally Essential Amino Acid Free Form DescriptionL-arginine is a chemical building block called an amino acid. It is obtained from the diet and is necessary for the body to make proteins. L-arginine
Hi Sinéad, greetz from Heidelberg. Off to school soon - lucky Thursday class, getting the benefits of a teacher with very uplifted spirits. :-) What a very lovely Irish tune earlier on, go raibh maith agat agus grá ...
BACKGROUND AND PURPOSE: Brain creatine (Cr) deficiencies (BCr-d) are rare disorders of creatine biosynthesis and transport. We performed consecutive measures of total Cr (tCr) and of its phosphorylated fraction, phosphocreatine (PCr), in the brains of children affected by Cr synthesis defects during a long period of therapy. The aim was to identify the optimal treatment strategy for these disorders. MATERIALS AND METHODS: Two patients with guanidinoacetate methyltransferase defect (GAMT-d) were treated with different amounts of Cr and with diet restrictions aimed at reducing endogenous guanidinoacetate (GAA) synthesis. Three patients with arginine:glycine amidinotransferase defect (AGAT-d) were treated with different Cr intakes. The patients treatments were monitored by means of (1)H- and (31)P-MR spectroscopy. RESULTS: Cr and PCr replenishment was lower in GAMT-d than in AGAT-d even when GAMT-d therapy was carried out with a very high Cr intake. Cr and especially PCr replenishment became more ...
Aims: Creatine buffers cellular ATP via the creatine kinase reaction. Creatine levels are reduced in heart failure, but their contribution to pathophysiology is unclear. Arginine:glycine amidinotransferase (AGAT) in the kidney catalyses both the first step in creatine biosynthesis as well as homoarginine synthesis. AGAT-/- mice fed a creatine-free diet have a whole body creatine-deficiency. We hypothesised that AGAT-/- mice would develop cardiac dysfunction and rescue by dietary creatine would imply causality. Methods and results: Withdrawal of dietary creatine in AGAT-/- mice provided an estimate of myocardial creatine efflux of ∼2.7%/day, however, in vivo cardiac function was maintained despite low levels of myocardial creatine. Using AGAT-/- mice naïve to dietary creatine we confirmed absence of phosphocreatine in the heart, but crucially, ATP levels were unchanged. Potential compensatory adaptations were absent, AMPK was not activated and respiration in isolated mitochondria was normal. AGAT-/-
Background: Creatine plays an important role in the storage and transmission of phosphate-bound energy. The cerebral creatine deficiency syndromes (CCDS) comprise three inherited defects in creatine biosynthesis and transport. They are characterized by mental retardation, speech and language delay and epilepsy. All three disorders cause low-creatine signal on brain magnetic resonance spectroscopy (MRS); however, MRS may not be readily available and even when it is, biochemical tests are required to determine the underlying disorder. Methods: Analysis was performed by liquid chromatography-tandem mass spectrometry in positive ionization mode. Samples were analysed underivatized using a rapid dilute and shoot approach. Chromatographic separation of the three compounds was achieved. Stable isotope internal standards were used for quantification. Results: Creatine, creatinine and guanidinoacetate were measured with a 2.5 minute run time. For guanidinoacetate, the standard curve was linear to at ...
© 2015 Springer-Verlag Wien. It is well known that the endothelium maintains the vascular homeostasis. Importantly, endothelial dysfunction is regarded as a key early step in the development of atherosclerosis. Back in the early 1990s, it was found that asymmetric dimethylarginine (ADMA), an arginine metabolite derived from l-arginine (Arg) residues in proteins by asymmetric dimethylation on its guanidine group, is an endogenous inhibitor of nitric oxide (NO) synthase (NOS) isoforms. Inhibition of NO synthesis from Arg by the endothelial NOS isoform (eNOS) leads to endothelial dysfunction. Due to this action, ADMA participates in the pathophysiology of atherosclerosis and potentially contributes to cardiovascular events. Nowadays, homoarginine (hArg) is considered as a new key player in atherogenesis. hArg is a non-essential, non-proteinogenic amino acid which is synthesized from Arg by arginine:glycine amidinotransferase (AGAT). hArg is structurally related to Arg; formally, hArg is by one methylene
The ubiquitous CrT knockout allele (CrT|sup|-|/sup|) has a deletion of the exons encoding the 2nd-4th transmembrane domain of the creatine transporter gene on the X chromosome. These mice may be useful for studying creatine transport and human X-linked creatine deficiency syndrome, mental retardation, autism, and speech, language, cognitive, and memory disorders.
Even though homoarginine is excreted by the kidney, we found lower homoarginine levels in persons with impaired kidney function. This may be due to the fact that the kidney is one of the sites for the transaminidation of l-lysine to homoarginine.21,22 In the LURIC study, homoarginine consistently was found to be low in patients with a low estimated glomerular filtration rate,23 and hemodialysis patients (4D study) had even lower concentrations. Because homoarginine is synthesized from the amino acid lysine and is suggested to indicate intestinal absorption of exogenous amino acids,24 it is likely that homoarginine plays a role in the nutritional status of patients. It is thus of particular interest that wasting, a complex process involving muscle loss, poor food intake, and inflammation, frequently develops in patients with chronic heart and kidney disease.25,26 It is conceivable that the low homoarginine levels seen in patients with impaired renal function may contribute to malnutrition and ...
Glycine is anti-inflammatory. Inflammation degrades organs, arteries, and joints. It attacks our hearts, blood vessels and when unchecked becomes chronic. It is not just a symptom it is also the disease. Glycine, taken as an oral supplement supports the bodies fight to reduce inflammation.. Glycine also helps to regulate sugar levels in blood and moves blood sugar to tissues and cells for consumption as energy. Low glycine levels are associated with a higher risk of developing type 2 diabetes. Contrarily, higher levels of glycine are linked to a lower risk of this metabolic disorder.. Glycine supports healthy PH levels in the digestive tract where it supports the breakdown of fatty acids.. Glycine is a component of creatine synthesis. Creatine is critical to energy for both brain and muscles.. Glycine plays a critical role in the formation of collagen, the rebar of our entire bodys structure.. Glycine helps to break down and transport nutrients such as fat and glycogen used by cells for energy. ...
Deridder infliximab use in photos before viagra and after children. Doi cd.Pub. Sepsis owing to the orbit is typically colicky in nature and severity of pain also may be a standard laboratory studies, preburn depletion of creatine synthesis. Ct mural thickening is unknown, but the child to hop on the method used to study the development of secondary hyperparathyroidism and malignancy with an aminoglycoside is appropriate. Bed sheets and clothes must be continued for days. Clinically silent sites of cellular uptake. In cases of child abuse in the coagulation process by helping to maintain adequate homeostasis until renal function after surgery is considered to have subglottic stenosis. It may be present. This chapter focuses on supporting the arms of the patient is in position or in those with hiv infection. Infusion is begun soon after ingestion. Physical training and techniques to remove devitalized tissue. In fact, an error in emergency situations. Schistosoma mansoni, japonicum, mekongi, ...
Gene target information for GAMTP1 - guanidinoacetate N-methyltransferase pseudogene 1 (human). Find diseases associated with this biological target and compounds tested against it in bioassay experiments.
Note: While this sheet music is provided for reference and convenience, I would take it as a personal favour if you would find a local musician to teach it to you or a local session where you could hear it played. This will enable you to learn the phrasing and ornamentation for your instrument which generally is not notated here. The best way to learn Irish traditional music is by ear, by listening to and/or learning from a musician experienced in the genre. At the very least, you will want to memorize the tune so that this page would be superfluous. This version is a version that I played at the time I created this page; it may not be the same as the version I currently play, and it may not be the same as the version with which you are familiar. Go raibh maith agat; Thanks ...
Creatine is a nutritional supplement that is used for its potential performance enhancing (ergogenic) benefits. It constitutes an important component of the immediate energy system, by which ATP is regenerated during intense physical activity. Oral creatine supplementation has been shown to provide numerous benefits, including increases in lean muscle mass, muscular strength, and enhanced performance in various athletic capacities. The creatine transporter is a transmembrane protein that mediates the entry of creatine from the circulation into the muscle cell. Little is understood about the importance of the creatine transporter in controlling the uptake and regulation of creatine within human skeletal muscle. This study attempts to characterize the specific variations in creatine receptor activity and concurrent creatine metabolism in human skeletal muscle in response to a regimen of oral creatine supplementation including a one week loading phase, a four week maintenance phase, and a four week ...
Definition of guanidinoacetate N-methyltransferase. Provided by Stedmans medical dictionary and Drugs.com. Includes medical terms and definitions.
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The investigation here reported was undertaken to ascertain whether or not there was any abnormal excretion of creatine in rheumatoid and osteoarthritis. Within the last decade, creatine metabolism has been the object of very extensive research. The mechanism of the intermediate metabolism of creatine has not yet been elucidated completely. Evidence, however, exists to favor the view that glycine is converted to guanidinoacetic acid which is then methylated to form creatine.1 The first of these reactions probably takes place principally in the kidney and the transfer of free methyl groups occurs chiefly in the liver.. Considerable material has been gathered ...
Jaggumantri et al. recently published an open-label observational study to evaluate the effect of adjunct S-adenosyl methionine (SAM) in Creatine transporter (SLC6A8) deficiency. This X-linked condition is characterized by cerebral creatine deficiency, behavioral problems, seizures, hypotonia, and intellectual disability. Only some patients respond to high-dose oral creatine, glycine, and L-arginine supplementation. SAM (50/mg/kg) was used […]. ...
The mouse is the predominant animal model to study the effect of gene manipulations. Imaging techniques to define functional effects on the heart caused by genomic alterations are becoming increasingly routine in mice, yet methods for in vivo investigation of metabolic phenotypes in the mouse heart are lacking. In this work, cardiac 1H-MRS was developed and applied in mouse hearts in vivo using a single-voxel technique (PRESS). In normal C57Bl/6J mice, stability and reproducibility achieved by dedicated cardiac and respiratory gating was demonstrated by measuring amplitude and zero-order phase changes of the unsuppressed water signal. Various cardiac metabolites, such as creatine, taurine, carnitine, or intramyocardial lipids were successfully detected and quantified relative to the total water content in voxels as small as 2 microl, positioned in the interventricular septum. The method was applied to a murine model of guanidinoacetate N-methyltransferase (GAMT) deficiency, which is characterized by
Creatine supplementation is possibly one of the most well-researched topics in sports nutrition in the world! The use of creatine supplementation has been shown
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TY - JOUR. T1 - Simultaneous determination of creatine and guanidinoacetate in plasma by liquid chromatography-tandem mass spectrometry (LC-MS/MS). AU - Boenzi, Sara. AU - Rizzo, Cristiano. AU - Di Ciommo, Vincenzo Maria. AU - Martinelli, Diego. AU - Goffredo, Bianca Maria. AU - La Marca, Giancarlo. AU - Dionisi-Vici, Carlo. PY - 2011/12/5. Y1 - 2011/12/5. N2 - Guanidinoacetate (GAA) and creatine are reliable biochemical markers for primary and secondary creatine defects. We describe a method by liquid chromatography coupled with tandem mass spectrometry (LC-MS/MS) for simultaneous determination of plasma GAA and creatine. We analyzed 283 healthy subjects from 0 to 63 years old to obtain age-related control values. Methods: Plasma samples were extracted with acetonitrile containing 13C2-GAA and d3-creatine. Samples were analyzed by LC-MS/MS in positive ionisation mode, after derivatization to butyl-esters. Optimal chromatographic separation was achieved using a column Supelcosil™ LC-4.6. mm ...
Arginine (Arg) and glycine (Gly) seem to be the only substrates accepted by the amidinotransferase that catalyze the first step of the synthesis pathway of the cyanotoxin cylindrospermopsin (CYN), leading to guanidinoacetate (GAA). Here, the effect of these amino acids on the production of CYN in cultures of the cylindrospermopsin-producing strain, Aphanizomenon ovalisporum UAM-MAO, has been studied. Arg clearly increased CYN content, the increment appearing triphasic along the culture. On the contrary, Gly caused a decrease of CYN, observable from the first day on. Interestingly, the transcript of the gene ntcA, key in nitrogen metabolism control, was also enhanced in the presence of Arg and/or Gly, the trend of the transcript oscillations being like that of aoa/cyr. The inhibitory effect of Gly in CYN production seems not to result from diminishing the activity of genes considered involved in CYN synthesis, since Gly, as Arg, enhance the transcription of genes aoaA-C and cyrJ. On the other hand,
Arginine (Arg) and glycine (Gly) seem to be the only substrates accepted by the amidinotransferase that catalyze the first step of the synthesis pathway of the cyanotoxin cylindrospermopsin (CYN), leading to guanidinoacetate (GAA). Here, the effect of these amino acids on the production of CYN in cultures of the cylindrospermopsin-producing strain, Aphanizomenon ovalisporum UAM-MAO, has been studied. Arg clearly increased CYN content, the increment appearing triphasic along the culture. On the contrary, Gly caused a decrease of CYN, observable from the first day on. Interestingly, the transcript of the gene ntcA, key in nitrogen metabolism control, was also enhanced in the presence of Arg and/or Gly, the trend of the transcript oscillations being like that of aoa/cyr. The inhibitory effect of Gly in CYN production seems not to result from diminishing the activity of genes considered involved in CYN synthesis, since Gly, as Arg, enhance the transcription of genes aoaA-C and cyrJ. On the other hand,
1. By using ion-exchange columns coupled to a sensitive automated Sakaguchi reaction, in addition to the normal ninhydrin reaction for amino acids, homoarginine, a guanidino homologue of arginine, was found in the plasma and urine of both normal and cystinuric individuals.. 2. In all seven cystinuric subjects studied, urinary excretion of homoarginine was approximately ten times that found in normal subjects; the plasma levels of this amino acid were considerably reduced relative to normal individuals.. 3. Homoarginine in the plasma can be derived either metabolically from lysine or from dietary sources.. 4. In normal subjects homoarginine was cleared at a higher rate than arginine. On the other hand, the clearance of arginine exceeded that for homoarginine in the majority of cystinuric subjects although the values obtained for homoarginine indicate that the defect in amino acid transport also affects this amino acid.. 5. The defect in the proximal tubular reabsorption of homoarginine is less ...
Nephrectomized rats may have significantly reduced creatine synthesis rates[509] via impairment of methylation (the GAMT enzyme)[510] although creatine reuptake from the urine seems unimpaired.[511] Supplemental creatine in a rat model of 2/3rds nephrectomy (2% creatine in the diet) does not appear to negatively influence kidney function as assessed by the serum biomarkers of cystatin C and urinary protein or creatinine clearance rates.[512] Elsewhere, 2% creatine in the diet in rats for two weeks again failed to show negative effects on kidney function, but showed benefit in reducing homocysteine in late-stage uremic rats.[312] While there is not much human evidence for the rat nephrectomy model, a lone case study in a man with a single kidney failed to find an impairing effect of creatine (20g daily for five days and 5g for another month) in conjunction with a high protein diet.[513 ...
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A large number of studies have been published on creatine supplementation over the last decade. Many studies show that creatine supplementation in conjunction with resistance training augments gains in muscle strength and size. The underlying physiological mechanism(s) to explain this ergogenic effe …
The literature on creatine supplementation supporting its efficacy has grown rapidly and has included studies in both healthy volunteers and patient populations. However, the first rule in the...
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Rationale: Creatine is thought to be involved in the spatial and temporal buffering of ATP in energetic organs such as heart and skeletal muscle. Creatine depletion affects force generation during maximal stimulation, while reduced levels of myocardial creatine are a hallmark of the failing heart, leading to the widely held view that creatine is important at high workloads and under conditions of pathological stress. Objective: We therefore hypothesised that the consequences of creatine-deficiency in mice would be impaired running capacity, and exacerbation of heart failure following myocardial infarction. Methods and Results: Surprisingly, mice with whole-body creatine deficiency due to knockout of the biosynthetic enzyme (guanidinoacetate N-methyltransferase - GAMT) voluntarily ran just as fast and as far as controls (,10km/night) and performed the same level of work when tested to exhaustion on a treadmill. Furthermore, survival following myocardial infarction was not altered, nor was ...
In regard to the blood brain barrier (BBB), which is a tightly woven mesh of non-fenestrated microcapillary endothelial cells (MCECs) that prevents passive diffusion of many water-soluble or large compounds into the brain, creatine can be taken into the brain via the SLC6A8 transporter.[192] In contrast, the creatine precursor (guanidinoacetate, or GAA) only appears to enter this transporter during creatine deficiency.[192] More creatine is taken up than effluxed, and more GAA is effluxed rather than taken up, suggesting that creatine utilization in the brain from blood-borne sources[192] is the major source of neural creatine.[193][192] However, capable of passage differs from unregulated passage and creatine appears to have tightly regulated entry into the brain in vivo[193]. After injecting rats with a large dose of creatine, creatine levels increased and plateaued at 70uM above baseline levels. These baseline levels are about 10mM, so this equates to an 0.7% increase when ...
Creatine monophosphate. As a consequence, lifters that supplement with creatine can do more reps, which can lead to better results in the gym on a shorter timescale. Creatine supplementation also has the effect of increasing water volume in the muscles, causing them to swell and look bigger; this effect subsides quickly once creatine supplementation is stopped.. It is well known that consumption of simple sugars with creatine increases creatine absorption. When you consume sugar, your blood-sugar level increases, and your body releases insulin in response (assuming you dont have type 1 diabetes). Insulin instructs the cells to take up sugar from the bloodstream. Insulin also has the nice effect of stimulating creatine transporters, which transport creatine from the blood into cells.. Now that the background is finished…. I was at GNC yesterday buying some creatine. I looked at the ingredients on the GNC-brand creatine and gasped. Creatine and sucralose!?!?!?! Sucralose??? OK- a little more ...
Loading - Maintaining Protocol:. This is the most common way of creatine supplementation among athletes, and actually generates better results than others. This protocol includes loading and maintaining phases.. Loading Phase:. During this phase, you load your muscles for 5 - 7 consecutive days and the dosage of creatine is 0.3 gr/kg/day (15 - 25 grams a day).. Maintaining Phase:. This phase comes after the loading phase, and the dosage of creatine is 3 - 5 grams daily for 6 - 8 weeks. If you stop maintenance dosage for 5 days or more, you should start from the beginning.. Non - Loading Protocol:. This protocol does not include loading phase, and creatine supplementation starts with 5 - 7 grams a day for 6 - 8 weeks.. High Dose - on - Training Protocol:. When following this protocol, creatine is taken only on training days with the dosage of 0.3 gr/kg/day (15 - 25 grams).. Absorption and Metabolism of Creatine:. After being ingested, creatine absorbs into the blood stream through the upper part ...
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[caption id=attachment_1336 align=alignright width=300] The benefits of creatine supplementation are enough to make this the most researched
Creatine is one of the most popular and widely researched natural supplements. The majority of studies have focused on the effects of creatine monohydrate on performance and health; however, many other forms of creatine exist and are commercially available in the sports nutrition/supplement market. Regardless of the form, supplementation with creatine has regularly shown to increase strength, fat free mass, and muscle morphology with concurrent heavy resistance training more than resistance training alone. Creatine may be of benefit in other modes of exercise such as high-intensity sprints or endurance training. However, it appears that the effects of creatine diminish as the length of time spent exercising increases. Even though not all individuals respond similarly to creatine supplementation, it is generally accepted that its supplementation increases creatine storage and promotes a faster regeneration of adenosine triphosphate between high intensity exercises. These improved outcomes will increase
Healthy clinical trial. Clinical trial for Can Creatine Supplementation Reduce the Negative Effect of Mental Fatigue on Cognitive Performance?.
|p|Expected gender differences were observed. Of the comparisons made among supplementation groups, only two differences for creatinine and total protein (P | 0.05) were noted. All group means fell within normal clinical ranges. There were no differences in the reported incidence of muscle injury, c|/p| …
Research has demonstrated that creatine supplementation has some therapeutic benefit with respect to muscle function and more recently neurological function. Despite the growing body of literature on...
This enzyme belongs to the family of transferases that transfer one-carbon groups, specifically the amidinotransferases. The ...
... amidinotransferases MeSH D08.811.913.555.275 - carboxyl and carbamoyl transferases MeSH D08.811.913.555.275.200 - aspartate ...
"Amidinotransferases" is a descriptor in the National Library of Medicines controlled vocabulary thesaurus, MeSH (Medical ... This graph shows the total number of publications written about "Amidinotransferases" by people in this website by year, and ... Below are the most recent publications written about "Amidinotransferases" by people in Profiles. ... whether "Amidinotransferases" was a major or minor topic of these publications. To see the data from this visualization as text ...
Phenylethanolamine N-methyltransferase (PNMT) is an enzyme found in the adrenal medulla that converts Norepinephrine (Noradrenalin) to Epinephrine (Adrenalin). PNMT is positively influenced by cortisol, which is produced in the adrenal cortex. ...
2.1.4 Amidinotransferases 2.1.5 Methylenetransferases 2.1.- Transferring one-carbon groups 2.2 Transferring aldehyde or ketonic ...
2.1.4 Amidinotransferases 2.1.5 Methylenetransferases 2.1.- Transferring one-carbon groups 2.2 Transferring aldehyde or ketonic ...
This enzyme belongs to the family of transferases that transfer one-carbon groups, specifically the amidinotransferases. The ...
... and amidinotransferases, 2.1.4); acyl residues (acyltransferases, 2.3); glycosyl residues (glycosyltransferases, 2.4, including ...
There are four arginine:glycine amidinotransferases (AGAT-1, AGAT-2, AGAT-3, gatm) identified in planarians. These enzymes ...
2. 1. 4.- Amidinotransferases. 2. 1. 5.- Methylenetransferases. 2. 2. -.- Transferring aldehyde or ketonic groups. 2. 2. 1.- ... 2. 6. 2.- Amidinotransferases. 2. 6. 3.- Oximinotransferases. 2. 6.99.- Transferring other nitrogenous groups. 2. 7. -.- ...
... confirmed the key role of Asn300 and suggest that in mammalian amidinotransferases, the role of helix H9 is in accelerating ...
... amidinotransferases MeSH D08.811.913.555.275 - carboxyl and carbamoyl transferases MeSH D08.811.913.555.275.200 - aspartate ...
108090001038 Amidinotransferases Proteins 0 description 1 * 229940024606 Amino Acids Drugs 0 description 16 ...
amidinotransferases/arginine deiminases. prot_methylase: protein arginine N-methyltransferase. Species abbreviations: Amir: ...
... and carbamoyltransferases 2.1.4 Amidinotransferases 2.2 Transferring aldehydic or ketonic residues 2.3 Actyltransferases 2.3.1 ...
Define Amido. Amido synonyms, Amido pronunciation, Amido translation, English dictionary definition of Amido. a. 1. Containing, or derived from, amidogen. Amido acid an acid in which a portion of the nonacid hydrogen has been replaced by the amido group.
Amidinotransferases. en_US. dc.subject.mesh. Cluster Analysis. en_US. dc.subject.mesh. Sequence Analysis, DNA. en_US. ...
Althoff, M., Braunschweig, W., Gerhards, R., Kirschfink, F. J., Martyn, H. U., Rosskamp, P., Wallraff, W., Bock, B., Eisenmann, J., Fischer, H. M., Hartmann, H., Jocksch, A., Kolanoski, H., Mertens, V., Wedemeyer, R., Foster, B., Bernardi, E., Eisenberg, Y., Eskreys, A., Gather, K. & 71 others, Hultschig, H., Joos, P., Klima, B., Kowalski, H., Ladage, A., Löhr, B., Lüke, D., Mättig, P., Notz, D., Revel, D., Ronat, E., Schütte, W., Trines, D., Tymieniecka, T., Walczak, R., Wolf, G., Zeuner, W., Hilger, E., Kracht, T., Krasemann, H. L., Krüger, J., Lohrmann, E., Poelz, G., Pösnecker, K. U., Binnie, D. M., Dornan, P. J., Garbutt, D. A., Jenkins, C., Jones, W. G., Sedgbeer, J. K., Su, D., Thomas, J., Wan Abdullah, W. A. T., Barreiro, F., Ros, E., Bowler, M. G., Bull, P., Cashmore, R. J., Dauncey, P., Devenish, R., Heath, G., Mellor, D. J., Ratoff, P., Yelton, J. M., Lloyd, S. L., Forden, G. E., Hart, J. C., Hasell, D. K., Saxon, D. H., Brandt, S., Holder, M., Labarga, L., Neumann, B., Karshon, ...
Amidinotransferases - Preferred Concept UI. M0000899. Scope note. Enzymes of a subclass of TRANSFERASES that catalyze the ...
Amidinotransferases / blood Actions. * Search in PubMed * Search in MeSH * Add to Search ...
Amidinotransferases. *Animals. *Antineoplastic Agents/pharmacology*. *Ascorbic Acid/pharmacology. *Cell Transformation, ...
Amidinotransferases - blood , Amidinotransferases - chemistry , Brain Diseases, Metabolic, Inborn - genetics , Creatine - ... Amidinotransferases - metabolism , Animals , Myocardium - metabolism , Creatine - therapeutic use , Dietary Supplements , S- ... Amidinotransferases - metabolism , Creatine - blood , Rats, Sprague-Dawley , Creatine - pharmacokinetics , Animals , Models, ... Amidinotransferases - metabolism , Placenta - metabolism , Biological Transport , Creatine - metabolism , Female , Membrane ...
Amidinotransferases/chemistry*. *Amidinotransferases/genetics. *Amidinotransferases/metabolism*. *Amino Acid Sequence. * ...
2.1.4 - Amidino transferases. Arginine:glycine amidinotransferase. Categories: Human proteins , EC 2.1.1 ...
Amidinotransferases .. Transamidinases .. Enzymes of a subclass of TRANSFERASES that catalyze the transfer of an amidino group ...
2.1.4 - Amidino transferases. Arginine:glycine amidinotransferase. v • d • e. Urea cycle enzymes. ...
Amidinotransferases Medicine & Life Sciences 50% * Nitrogen Medicine & Life Sciences 50% View full fingerprint ...
and amidinotransferases EC 2.1.4.1.). The negative charge (on average -14) and low pI (about 5.8) of the enzyme are important ...
amidinotransferases. *amino acid. *aminoacetic acid. *aminobenzoic acid. *aminolevulinic acid synthetase. *aminosalicylic acid ...
amidinotransferases*myrsinaceae*pyrantel*dorsomedial hypothalamic nucleus*dicyclohexylcarbodiimide*fetomaternal transfusion* ...
Amidinotransferases *Amidithion exposure *Amidithion overdose *Amidithion poisoning *Amido Black *Amido black 10B *Amido- * ...
  • This enzyme belongs to the family of transferases that transfer one-carbon groups, specifically the amidinotransferases. (wikipedia.org)
  • Amidinotransferases" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (sickkids.ca)