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Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
arginine + 3-aminopropionic acid
3-guanidinopropionic acid + ornithine
arginine + 4-aminobutyric acid
4-guanidinobutyric acid + ornithine
arginine + 5-aminovaleric acid
5-guanidinovaleric acid + ornithine
-
-
-
-
?
arginine + delta-aminovaleric acid
delta-guanidinovaleric acid + ornithine
-
-
-
-
?
arginine + ethanolamine
2-guanidinoethanol + ornithine
arginine + gamma-aminobutyric acid
gamma-guanidinobutyric acid + ornithine
-
-
-
-
?
arginine + glycine
?
-
assay at pH 7.4, 37°C
-
-
?
arginine + hydroxylamine
ornithine + hydroxyguanidine
arginine + lysine
homoarginine + ornithine
-
-
-
-
?
arginine + ornithine
ornithine + arginine
arginine + taurine
2-guanidinoethanesulfonic acid + ornithine
canavanine + canaline
canaline + canavanine
canavanine + glycine
guanidinoacetate + canaline
canavanine + hydroxylamine
canaline + hydroxyguanidine
canavanine + ornithine
arginine + canaline
glycine + guanidinoacetate
guanidinoacetate + glycine
guanidinoacetate + canaline
glycine + canavanine
guanidinoacetate + glycine
?
guanidinoacetate + hydroxylamine
hydroxyguanidine + glycine
guanidinoacetate + ornithine
glycine + arginine
homoarginine + glycine
homoornithine + guanidinoacetate
hydroxyguanidine + glycine
hydroxylamine + guanidinoacetate
-
-
-
-
?
hydroxylamine + glycine
L-ornithine + hydroxyguanidine
L-alanine + glycine
?
-
-
-
-
?
L-aminobutyric acid + glycine
?
-
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
L-norvaline + glycine
?
-
-
-
-
?
L-ornithine + guanidinoacetate
L-arginine + glycine
additional information
?
-
arginine + 3-aminopropionic acid
3-guanidinopropionic acid + ornithine
-
-
-
-
?
arginine + 3-aminopropionic acid
3-guanidinopropionic acid + ornithine
-
-
-
-
?
arginine + 4-aminobutyric acid
4-guanidinobutyric acid + ornithine
-
-
-
-
?
arginine + 4-aminobutyric acid
4-guanidinobutyric acid + ornithine
-
-
-
-
?
arginine + 4-aminobutyric acid
4-guanidinobutyric acid + ornithine
-
-
-
-
?
arginine + ethanolamine
2-guanidinoethanol + ornithine
-
-
-
-
?
arginine + ethanolamine
2-guanidinoethanol + ornithine
-
-
-
-
?
arginine + ethanolamine
2-guanidinoethanol + ornithine
-
-
-
-
?
arginine + ethanolamine
2-guanidinoethanol + ornithine
-
-
-
-
?
arginine + hydroxylamine
ornithine + hydroxyguanidine
-
-
-
-
?
arginine + hydroxylamine
ornithine + hydroxyguanidine
-
-
-
-
?
arginine + ornithine
ornithine + arginine
-
-
-
-
?
arginine + ornithine
ornithine + arginine
-
-
-
-
?
arginine + taurine
2-guanidinoethanesulfonic acid + ornithine
-
-
-
-
?
arginine + taurine
2-guanidinoethanesulfonic acid + ornithine
-
-
-
-
?
canavanine + canaline
canaline + canavanine
-
-
-
-
?
canavanine + canaline
canaline + canavanine
-
-
-
-
?
canavanine + canaline
canaline + canavanine
-
-
-
-
?
canavanine + glycine
guanidinoacetate + canaline
-
-
-
-
r
canavanine + glycine
guanidinoacetate + canaline
-
-
-
-
r
canavanine + glycine
guanidinoacetate + canaline
-
-
-
-
r
canavanine + glycine
guanidinoacetate + canaline
-
-
-
-
r
canavanine + hydroxylamine
canaline + hydroxyguanidine
-
-
-
-
?
canavanine + hydroxylamine
canaline + hydroxyguanidine
-
-
-
-
?
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
Felis domestica
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
Lacerta sp.
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
485961, 485964, 485965, 485966, 485967, 485968, 485969, 485970, 485971, 485972, 485974, 485975 -
-
r
canavanine + ornithine
arginine + canaline
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
485960, 485961, 485962, 485963, 485964, 485965, 485966, 485967, 485968, 485971, 485973, 485974, 485975, 485976, 485978 -
-
r
glycine + guanidinoacetate
guanidinoacetate + glycine
-
-
-
-
r
glycine + guanidinoacetate
guanidinoacetate + glycine
-
-
-
-
r
guanidinoacetate + canaline
glycine + canavanine
-
-
-
-
?
guanidinoacetate + canaline
glycine + canavanine
-
-
-
-
?
guanidinoacetate + glycine
?
-
-
-
?
guanidinoacetate + glycine
?
-
-
-
?
guanidinoacetate + hydroxylamine
hydroxyguanidine + glycine
-
-
-
-
?
guanidinoacetate + hydroxylamine
hydroxyguanidine + glycine
-
-
-
-
?
guanidinoacetate + hydroxylamine
hydroxyguanidine + glycine
-
-
-
-
?
guanidinoacetate + ornithine
glycine + arginine
-
-
-
-
?
guanidinoacetate + ornithine
glycine + arginine
-
-
-
-
?
homoarginine + glycine
homoornithine + guanidinoacetate
-
-
-
-
r
homoarginine + glycine
homoornithine + guanidinoacetate
-
-
-
-
r
homoarginine + glycine
homoornithine + guanidinoacetate
-
-
-
-
r
homoarginine + glycine
homoornithine + guanidinoacetate
-
-
-
-
r
hydroxylamine + glycine
L-ornithine + hydroxyguanidine
-
-
-
?
hydroxylamine + glycine
L-ornithine + hydroxyguanidine
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
L-ornithine is formed in the presence of only a single substrate, arginine, and its production therefore does not require the presence of the second substrate, glycine
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
L-ornithine is formed in the presence of only a single substrate, arginine, and its production therefore does not require the presence of the second substrate, glycine
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
Felis domestica
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
nitrogen metabolism, putrescine biosynthesis
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
first reaction in the de novo biosynthesis of creatine
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
first reaction in the de novo biosynthesis of creatine
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
enzyme is involved in biosynthesis of creatine. Patients with AGAT deficiency show mental and motor retardation and severe delay in speech development. Both creatine and guanidinoacetate are decreased in body fluids of AGAT-deficient patients
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
Lacerta sp.
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
485961, 485964, 485965, 485966, 485967, 485968, 485969, 485970, 485971, 485972, 485974, 485975 -
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
first reaction in the de novo biosynthesis of creatine
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
first reaction in the de novo biosynthesis of creatine
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
485960, 485961, 485962, 485963, 485964, 485965, 485966, 485967, 485968, 485971, 485973, 485974, 485975, 485976, 485978 -
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
485960, 485961, 485962, 485963, 485964, 485965, 485966, 485967, 485968, 485971, 485973, 485974, 485975, 485976, 485978 -
-
r
additional information
?
-
narrow substrate specificity, no activity with L-homoarginine, agmatine, L-canavanine, guanidine hydrochloride, urea, gamma-guanidinobutyric acid, and beta-guanidinoproprionic acid as amidino group donors, or L-alanine, beta-alanine, gamma-aminobutyric acid, ethanolamine, taurine, L-lysine, alpha-amino-oxyacetic acid and L-norvaline as amidino group acceptors, overview
-
-
?
additional information
?
-
no activity with creatine, L-homoarginine, and L-canavanine by the wild-type enzyme, while mutants F245N, S247M, and F245N/S247M are also active L-homoarginine and L-canavanine, mutant F254N also slightly with creatine, overview
-
-
?
additional information
?
-
narrow substrate specificity, no activity with L-homoarginine, agmatine, L-canavanine, guanidine hydrochloride, urea, gamma-guanidinobutyric acid, and beta-guanidinoproprionic acid as amidino group donors, or L-alanine, beta-alanine, gamma-aminobutyric acid, ethanolamine, taurine, L-lysine, alpha-amino-oxyacetic acid and L-norvaline as amidino group acceptors, overview
-
-
?
additional information
?
-
-
narrow substrate specificity, no activity with L-homoarginine, agmatine, L-canavanine, guanidine hydrochloride, urea, gamma-guanidinobutyric acid, and beta-guanidinoproprionic acid as amidino group donors, or L-alanine, beta-alanine, gamma-aminobutyric acid, ethanolamine, taurine, L-lysine, alpha-amino-oxyacetic acid and L-norvaline as amidino group acceptors, overview
-
-
?
additional information
?
-
no activity with creatine, L-homoarginine, and L-canavanine by the wild-type enzyme, while mutants F245N, S247M, and F245N/S247M are also active L-homoarginine and L-canavanine, mutant F254N also slightly with creatine, overview
-
-
?
additional information
?
-
-
homoarginine, alpha-amino-gamma-guanidinobutyric acid and alpha-amino-beta-guanidinopropionic acid are not substrates
-
-
?
additional information
?
-
-
L-glutamic acid, L-aspartic acid and histidine are no substrates
-
-
?
additional information
?
-
-
L-glutamic acid, L-aspartic acid and histidine are no substrates
-
-
?
additional information
?
-
-
possible amidino group acceptors gamma-aminobutyric acid, beta-alanine, hydroxylamine
-
-
?
additional information
?
-
-
lysine will not substitute for ornithine
-
-
?
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
L-arginine + glycine
L-ornithine + guanidinoacetate
L-ornithine + guanidinoacetate
L-arginine + glycine
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
Felis domestica
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
nitrogen metabolism, putrescine biosynthesis
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
first reaction in the de novo biosynthesis of creatine
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
first reaction in the de novo biosynthesis of creatine
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
enzyme is involved in biosynthesis of creatine. Patients with AGAT deficiency show mental and motor retardation and severe delay in speech development. Both creatine and guanidinoacetate are decreased in body fluids of AGAT-deficient patients
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
Lacerta sp.
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
485961, 485964, 485965, 485966, 485967, 485968, 485969, 485970, 485971, 485972, 485974, 485975 -
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
first reaction in the de novo biosynthesis of creatine
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
first reaction in the de novo biosynthesis of creatine
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
485960, 485961, 485962, 485963, 485964, 485965, 485966, 485967, 485968, 485971, 485973, 485974, 485975, 485976, 485978 -
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
485960, 485961, 485962, 485963, 485964, 485965, 485966, 485967, 485968, 485971, 485973, 485974, 485975, 485976, 485978 -
-
r
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Acute Kidney Injury
Interrelation of urinary and plasma levels of guanidinoacetic acid with alteration in renal activity of glycine amidinotransferase in acute renal failure rats.
Acute Kidney Injury
[Activity of glycine amidinotransferase and its isoforms in dog kidney tissue normally and in acute renal insufficiency]
Breast Neoplasms
Creatine-mediated crosstalk between adipocytes and cancer cells regulates obesity-driven breast cancer.
Carcinoma
A short review on creatine-creatine kinase system in relation to cancer and some experimental results on creatine as adjuvant in cancer therapy.
Carcinoma
Enzymes of creatine biosynthesis, arginine and methionine metabolism in normal and malignant cells.
Dent Disease
Novel Fanconi renotubular syndromes provide insights in proximal tubule pathophysiology.
Diabetes Mellitus
[Study of impaired metabolism of guanidinoacetic acid in uremia--the compensatory role of the pancreas in guanidinoacetic acid synthesis]
Fanconi Syndrome
Glycine Amidinotransferase (GATM), Renal Fanconi Syndrome, and Kidney Failure.
Genetic Diseases, Inborn
LC-MS/MS measurements of urinary guanidinoacetic acid and creatine: Method optimization by deleting derivatization step.
Glomerulonephritis
[Glycine amidinotransferase activity of the blood and urine of children with acute streptococcal glomerulonephritis]
glycine amidinotransferase deficiency
Arginine-Glycine Amidinotransferase Deficiency and Functional Characterization of Missense Variants in GATM.
glycine amidinotransferase deficiency
Arginine:glycine amidinotransferase (AGAT) deficiency in a newborn: early treatment can prevent phenotypic expression of the disease.
glycine amidinotransferase deficiency
Arginine:glycine amidinotransferase deficiency: a treatable metabolic encephalomyopathy.
glycine amidinotransferase deficiency
Arginine:glycine amidinotransferase deficiency: the third inborn error of creatine metabolism in humans.
glycine amidinotransferase deficiency
Cerebral creatine deficiency syndromes: clinical aspects, treatment and pathophysiology.
glycine amidinotransferase deficiency
Creatine and creatine deficiency syndromes: biochemical and clinical aspects.
glycine amidinotransferase deficiency
Creatine metabolism in urea cycle defects.
glycine amidinotransferase deficiency
Developmental progress and creatine restoration upon long-term creatine supplementation of a patient with arginine:glycine amidinotransferase deficiency.
glycine amidinotransferase deficiency
Diagnostic enzyme assay that uses stable-isotope-labeled substrates to detect L-arginine:glycine amidinotransferase deficiency.
glycine amidinotransferase deficiency
Fifteen-year follow-up of Italian families affected by arginine glycine amidinotransferase deficiency.
glycine amidinotransferase deficiency
Homoarginine- and Creatine-Dependent Gene Regulation in Murine Brains with l-Arginine:Glycine Amidinotransferase Deficiency.
glycine amidinotransferase deficiency
Inborn errors of creatine metabolism and epilepsy: clinical features, diagnosis, and treatment.
glycine amidinotransferase deficiency
L-arginine:glycine amidinotransferase deficiency protects from metabolic syndrome.
glycine amidinotransferase deficiency
LC-MS/MS measurements of urinary guanidinoacetic acid and creatine: Method optimization by deleting derivatization step.
guanidinoacetate n-methyltransferase deficiency
Cerebral creatine deficiency syndromes: clinical aspects, treatment and pathophysiology.
guanidinoacetate n-methyltransferase deficiency
Creatine and creatine deficiency syndromes: biochemical and clinical aspects.
guanidinoacetate n-methyltransferase deficiency
Creatine metabolism in urea cycle defects.
guanidinoacetate n-methyltransferase deficiency
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.
guanidinoacetate n-methyltransferase deficiency
LC-MS/MS measurements of urinary guanidinoacetic acid and creatine: Method optimization by deleting derivatization step.
Gyrate Atrophy
Inhibition of arginine-glycine amidinotransferase by ornithine. A possible mechanism for the muscular and chorioretinal atrophies in gyrate atrophy of the choroid and retina with hyperornithinemia.
Gyrate Atrophy
Ornithine delta-aminotransferase activity in retina and other tissues.
Heart Failure
Myocardial expression of the arginine:glycine amidinotransferase gene is elevated in heart failure and normalized after recovery: potential implications for local creatine synthesis.
Intellectual Disability
Arginine:glycine amidinotransferase (AGAT) deficiency in a newborn: early treatment can prevent phenotypic expression of the disease.
Intellectual Disability
Creatine depletion in a new case with AGAT deficiency: clinical and genetic study in a large pedigree.
Intellectual Disability
Fifteen-year follow-up of Italian families affected by arginine glycine amidinotransferase deficiency.
Intellectual Disability
Inborn errors of creatine metabolism and epilepsy: clinical features, diagnosis, and treatment.
Kidney Failure, Chronic
Impaired metabolism of guanidinoacetic acid in uremia.
Language Disorders
Inborn errors of creatine metabolism and epilepsy: clinical features, diagnosis, and treatment.
Metabolic Syndrome
Differential regulation of AMPK activation in leptin- and creatine-deficient mice.
Metabolic Syndrome
L-arginine:glycine amidinotransferase deficiency protects from metabolic syndrome.
Metabolism, Inborn Errors
Health implications of creatine: can oral creatine supplementation protect against neurological and atherosclerotic disease?
Metabolism, Inborn Errors
[Cerebral creatine transporter deficiency: an infradiagnosed neurometabolic disease]
Muscular Diseases
Creatine deficiency syndrome. A treatable myopathy due to arginine-glycine amidinotransferase (AGAT) deficiency.
Muscular Diseases
Does reduced creatine synthesis protect against statin myopathy?
Muscular Diseases
Muscle phenotype of AGAT- and GAMT-deficient mice after simvastatin exposure.
Muscular Diseases
Statins and skeletal muscles toxicity: from clinical trials to everyday practice.
Muscular Dystrophies
Reduced renal arginine-glycine transamidinase activity in myotonic goats and in patients with myotonic muscular dystrophy.
Myotonic Dystrophy
Arginine-glycine amidinotransferase activity in myotonic dystrophy.
Neoplasm Metastasis
Creatine promotes cancer metastasis through activation of Smad2/3.
Neoplasms
Creatine-mediated crosstalk between adipocytes and cancer cells regulates obesity-driven breast cancer.
Neoplasms
Enzymes of creatine biosynthesis, arginine and methionine metabolism in normal and malignant cells.
Nephrosis, Lipoid
[Glycine transamidinase (ADT) activity in the serum and urine of children with lipoid nephrosis]
Pancreatitis
[Repression and depression of glycine amidinotransferase in the rat pancreas at different stages of the development of pancreatitis]
Renal Insufficiency
Genetic mitochondrial glycine amidinotransferase protein aggregate formation triggers microparticle sensing and kidney failure.
Renal Insufficiency
Glycine Amidinotransferase (GATM), Renal Fanconi Syndrome, and Kidney Failure.
Renal Insufficiency
Interrelation of urinary and plasma levels of guanidinoacetic acid with alteration in renal activity of glycine amidinotransferase in acute renal failure rats.
Renal Insufficiency
[Study of impaired metabolism of guanidinoacetic acid in uremia--the compensatory role of the pancreas in guanidinoacetic acid synthesis]
Sarcoma
A short review on creatine-creatine kinase system in relation to cancer and some experimental results on creatine as adjuvant in cancer therapy.
Sarcoma
Enzymes of creatine biosynthesis, arginine and methionine metabolism in normal and malignant cells.
Sarcoma 180
A short review on creatine-creatine kinase system in relation to cancer and some experimental results on creatine as adjuvant in cancer therapy.
Sarcoma 180
Enzymes of creatine biosynthesis, arginine and methionine metabolism in normal and malignant cells.
Stroke
Homoarginine Levels Are Regulated by L-Arginine:Glycine Amidinotransferase and Affect Stroke Outcome: Results From Human and Murine Studies.
Stroke
Homoarginine- and Creatine-Dependent Gene Regulation in Murine Brains with l-Arginine:Glycine Amidinotransferase Deficiency.
Tuberous Sclerosis
Proteomes analysis reveals the involvement of autophagy in AD-like neuropathology induced by noise exposure and ApoE4.
Uremia
Impaired metabolism of guanidinoacetic acid in uremia.
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Structure and reaction mechanism of L-arginine:glycine amidinotransferase
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The comparative amino acid sequences, substrate specificities and gene or cDNA nucleotide sequences of some prokaryote and eukaryote amidinotransferases: implications for evolution
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Homo sapiens, Lampetra planeri, no activity in Streptomyces sp., Rattus norvegicus, Sus scrofa
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The ligand-induced structural changes of human L-arginine:glycine amidinotransferase. A mutational and crystallographic study
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Estrogen modulates the expression of L-arginine:glycine amidinotransferase in chick liver
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Gallus gallus, Gallus gallus (Q9I9K9), Homo sapiens, Rana sp., Rattus norvegicus, Sus scrofa
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Carducci, C.; Birarelli, M.; Leuzzi, V.; Carducci, C.; Battini, R.; Cioni, G.; Antonozzi, I.
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
Clin. Chem.
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Homo sapiens
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Lee, G.T.; Kim, W.J.; Cho, Y.D.
Polyamine synthesis in plants. Purification and properties of amidinotransferase from soybean (Glycine max) axes
Phytochemistry
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Glycine max
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Verhoeven, N.M.; Schor, D.S.M.; Roos, B.; Battini, R.; Stockler-Ipsiroglu, S.; Salomons, G.S.; Jakobs, C.
Diagnostic enzyme assay that uses stable-isotope-labeled substrates to detect L-arginine: glycine amidinotransferase deficiency
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Homo sapiens
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Verhoeven, N.M.; Salomons, G.S.; Jakobs, C.
Laboratory diagnosis of defects of creatine biosynthesis and transport
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Homo sapiens
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Wang, L.; Chen, D.; Zhang, Y.; Lin, Y.; Li, J.; Zhang, H.
Characterization of AGAT, GAMT and CT1 in amphioxus: implications for the evolutionary conservation of creatine metabolism related molecules at the invertebrate-to-vertebrate transition
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2008
Branchiostoma belcheri (Q0GU46)
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Braissant, O.; Cagnon, L.; Monnet-Tschudi, F.; Speer, O.; Wallimann, T.; Honegger, P.; Henry, H.
Ammonium alters creatine transport and synthesis in a 3D culture of developing brain cells, resulting in secondary cerebral creatine deficiency
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Rattus norvegicus (P50442)
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AGAT, GAMT and SLC6A8 distribution in the central nervous system, in relation to creatine deficiency syndromes: A review
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2008
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Expression and function of AGAT, GAMT and CT1 in the mammalian brain
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Enzymes of creatine biosynthesis, arginine and methionine metabolism in normal and malignant cells
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2008
Mus musculus
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Bourdelas, A.; Li, H.Y.; Carron, C.; Shi, D.L.
Dynamic expression pattern of distinct genes in the presomitic and somitic mesoderm during Xenopus development
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2009
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Brosnan, J.T.; Wijekoon, E.P.; Warford-Woolgar, L.; Trottier, N.L.; Brosnan, M.E.; Brunton, J.A.; Bertolo, R.F.
Creatine synthesis is a major metabolic process in neonatal piglets and has important implications for amino acid metabolism and methyl balance
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Muenchhoff, J.; Siddiqui, K.S.; Poljak, A.; Raftery, M.J.; Barrow, K.D.; Neilan, B.A.
A novel prokaryotic L-arginine:glycine amidinotransferase is involved in cylindrospermopsin biosynthesis
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Identification of two residues essential for the stringent substrate specificity and active site stability of the prokaryotic L-arginine:glycine amidinotransferase CyrA
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Choe, C.U.; Atzler, D.; Wild, P.S.; Carter, A.M.; Boeger, R.H.; Ojeda, F.; Simova, O.; Stockebrand, M.; Lackner, K.; Nabuurs, C.; Marescau, B.; Streichert, T.; Mueller, C.; Lueneburg, N.; De Deyn, P.P.; Benndorf, R.A.; Baldus, S.; Gerloff, C.; Blankenberg, S.; Heerschap, A.; Grant, P.J.; Magnus, T.; Zeller, T.; Isbrandt, D.; Schwedhelm, E.
Homoarginine levels are regulated by L-arginine:glycine amidinotransferase and affect stroke outcome: results from human and murine studies
Circulation
128
1451-1461
2013
Homo sapiens (P50440), Homo sapiens, Mus musculus (Q9D964), Mus musculus, Mus musculus C57BL6 (Q9D964)
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Jang, H.J.; Lee, M.O.; Kim, S.; Kim, T.H.; Kim, S.K.; Song, G.; Womack, J.E.; Han, J.Y.
Biallelic expression of the L-arginine:glycine amidinotransferase gene with different methylation status between male and female primordial germ cells in chickens
Poult. Sci.
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2013
Gallus gallus (Q9I9K9), Gallus gallus, Gallus gallus White leghorn (Q9I9K9), Gallus gallus Korean Oge (Q9I9K9)
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Faller, K.M.E.; Atzler, D.; McAndrew, D.J.; Zervou, S.; Whittington, H.J.; Simon, J.N.; Aksentijevic, D.; Ten Hove, M.; Choe, C.U.; Isbrandt, D.; Casadei, B.; Schneider, J.E.; Neubauer, S.; Lygate, C.A.
Impaired cardiac contractile function in arginine glycine amidinotransferase knockout mice devoid of creatine is rescued by homoarginine but not creatine
Cardiovasc. Res.
114
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2018
Mus musculus
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