Creatine pathway (WP5190)

Homo sapiens

In humans, creatine is synthesized in the liver, pancreas, kidney and brain. From arginine and glycine, guanidinoacetate and ornithine are formed. With S-adenosylmethionine and the help of GAMT, guanidinoacetate is converted into creatine. From the liver, pancreas, kidney and brain, creatine is exported to tissues such as skeletal muscle and brain, where it undergoes phosphorylation and serves as a short-term energy store. Creatine is transported to these tissues with the help of SLC6A8 transporter. Once formed, phosphocreatine and creatine undergo both a slow spontaneous reaction to form creatinine, which is excreted from the body via the urinary system. This pathway was inspired by Chapter 32 of the book of Blau (ISBN 3642403360 (978-3642403361) ed. 4).


Tim Zotti , Andra Waagmeester , Denise Slenter , Eric Weitz , and Friederike Ehrhart


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Homo sapiens


Inherited Metabolic Disorders (IMD) Pathways Rare Diseases


Disease Ontology

creatine transporter deficiency gyrate atrophy guanidinoacetate methyltransferase deficiency optic atrophy AGAT deficiency cerebral creatine deficiency syndrome Fanconi renotubular syndrome 1

Pathway Ontology

creatine metabolic pathway disease pathway guanidinoacetate methyltransferase deficiency pathway Fanconi syndrome pathway classic metabolic pathway gyrate atrophy pathway


Label Type Compact URI Comment
Arginine Metabolite chebi:32682
Creatinine Metabolite chebi:16737
S-adenosylmethionine Metabolite chebi:15414
Vitamin B6 Metabolite chebi:27306
Creatine Metabolite chebi:16919
Guanidinoacetate Metabolite chebi:16344
ADP Metabolite chebi:456216
Pyrroline-5-carboxylate Metabolite chebi:15893
ADP Metabolite chebi:456216
Creatine Metabolite chebi:16919
ATP Metabolite chebi:30616
Glutamate Metabolite chebi:14321
Glutamate-5-semialdehyde Metabolite wikidata:Q2823261
Proline Metabolite chebi:26271
Phosphocreatine Metabolite hmdb:HMDB0001511
ATP Metabolite chebi:30616
Ornithine Metabolite chebi:45453
S-Adenosylhomocysteine Metabolite chebi:16680
Glycine Metabolite hmdb:HMDB0000123
Creatinine Metabolite chebi:16737
2-oxoglutarate Metabolite chebi:16810
Phospho-guanidinoacetate Metabolite chebi:16034
Ornithine Metabolite chebi:45453
SLC6A8 GeneProduct ensembl:ENSG00000130821
GAMT GeneProduct ensembl:ENSG00000130005
GATM GeneProduct ensembl:ENSG00000171766 aka AGAT
OAT GeneProduct ensembl:ENSG00000065154
CK Protein uniprot:P12277
CK Protein uniprot:P12277


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