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Information on EC 2.1.4.1 - glycine amidinotransferase
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EC Tree
2.1.4.1 glycine amidinotransferaseIUBMB Comments
Canavanine can act instead of arginine.
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Word Map
- 2.1.4.1
- creatine
- guanidinoacetate
- homoarginine
-
slc6a8
- harg
- phosphocreatine
- medicine
-
creatine-deficient
-
transamidination
The enzyme appears in viruses and cellular organisms
Synonyms
l-arginine:glycine amidinotransferase, glycine amidinotransferase, arginine:glycine amidinotransferase, arginine-glycine amidinotransferase, glycine aminotransferase, arginine-glycine transamidinase, glycine transamidinase, 
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
AGAT
arginine-glycine amidinotransferase
-
-
-
-
arginine-glycine transamidinase
-
-
-
-
arginine:glycine amidinotransferase
CyrA
EC 2.6.2.1
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-
formerly
-
GAT
GATM
glycine amidinotransferase
glycine transamidinase
-
-
-
-
L-arginine:glycine amidinotransferase
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
L-arginine + glycine = L-ornithine + guanidinoacetate
canavanine can act instead of arginine
-
-
-
L-arginine + glycine = L-ornithine + guanidinoacetate
reaction and kinetic mechanisms, overview
L-arginine + glycine = L-ornithine + guanidinoacetate
reaction and kinetic mechanisms, overview
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-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
amidine group transfer
transamidination
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-
-
-
amidine group transfer
-
-
-
-
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PATHWAY SOURCE
PATHWAYS
BRENDA
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SYSTEMATIC NAME
IUBMB Comments
L-arginine:glycine amidinotransferase
Canavanine can act instead of arginine.
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CAS REGISTRY NUMBER
COMMENTARY
9027-35-4
-
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
arginine + 5-aminovaleric acid
5-guanidinovaleric acid + ornithine
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-
-
-
?
arginine + delta-aminovaleric acid
delta-guanidinovaleric acid + ornithine
-
-
-
-
?
arginine + gamma-aminobutyric acid
gamma-guanidinobutyric acid + ornithine
-
-
-
-
?
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
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-
-
-
?
arginine + 4-aminobutyric acid
4-guanidinobutyric acid + ornithine
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-
-
-
?
arginine + ethanolamine
2-guanidinoethanol + ornithine
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-
-
-
?
canavanine + glycine
guanidinoacetate + canaline
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-
-
-
r
canavanine + glycine
guanidinoacetate + canaline
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-
-
-
r
canavanine + ornithine
arginine + canaline
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-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
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-
485960, 485961, 485962, 485963, 485964, 485965, 485966, 485967, 485968, 485971, 485973, 485974, 485975, 485976, 485978
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-
r
guanidinoacetate + hydroxylamine
hydroxyguanidine + glycine
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-
-
-
?
guanidinoacetate + hydroxylamine
hydroxyguanidine + glycine
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-
-
-
?
homoarginine + glycine
homoornithine + guanidinoacetate
-
-
-
-
r
hydroxylamine + glycine
L-ornithine + hydroxyguanidine
-
-
-
?
hydroxylamine + glycine
L-ornithine + hydroxyguanidine
-
-
-
?
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
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-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
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-
-
?
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
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-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
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-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
-
nitrogen metabolism, putrescine biosynthesis
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-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
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r
L-arginine + glycine
L-ornithine + guanidinoacetate
first reaction in the de novo biosynthesis of creatine
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-
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
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-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
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-
-
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r
L-arginine + glycine
L-ornithine + guanidinoacetate
first reaction in the de novo biosynthesis of creatine
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-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
first reaction in the de novo biosynthesis of creatine
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r
L-arginine + glycine
L-ornithine + guanidinoacetate
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-
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r
L-arginine + glycine
L-ornithine + guanidinoacetate
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-
485960, 485961, 485962, 485963, 485964, 485965, 485966, 485967, 485968, 485971, 485973, 485974, 485975, 485976, 485978
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-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
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-
485960, 485961, 485962, 485963, 485964, 485965, 485966, 485967, 485968, 485971, 485973, 485974, 485975, 485976, 485978
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-
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
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-
?
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
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-
?
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
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-
?
additional information
?
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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
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?
additional information
?
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homoarginine, alpha-amino-gamma-guanidinobutyric acid and alpha-amino-beta-guanidinopropionic acid are not substrates
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?
additional information
?
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L-glutamic acid, L-aspartic acid and histidine are no substrates
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?
additional information
?
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L-glutamic acid, L-aspartic acid and histidine are no substrates
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?
additional information
?
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possible amidino group acceptors gamma-aminobutyric acid, beta-alanine, hydroxylamine
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-
?
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
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
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
-
-
-
-
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
-
-
-
-
r