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Literature summary for 2.1.4.1 extracted from

  • Braissant, O.; Henry, H.
    AGAT, GAMT and SLC6A8 distribution in the central nervous system, in relation to creatine deficiency syndromes: A review (2008), J. Inherit. Metab. Dis., 31, 230-239.
    View publication on PubMed

Application

Application Comment Organism
medicine creatine deficiency syndromes, either due to AGAT, GAMT or SLC6A8 deficiencies, lead to a complete absence, or a very strong decrease, of creatine within the brain Mammalia

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
L-arginine + glycine Mammalia
-
L-ornithine + guanidinoacetate
-
?

Organism

Organism UniProt Comment Textmining
Mammalia
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
Mammalia
-
central nervous system
-
Mammalia
-
kidney
-
Mammalia
-
liver
-
Mammalia
-
pancreas
-
Mammalia
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
L-arginine + glycine
-
Mammalia L-ornithine + guanidinoacetate
-
?

Synonyms

Synonyms Comment Organism
AGAT
-
Mammalia
arginine:glycine amidinotransferase
-
Mammalia
glycine amidinotransferase
-
Mammalia