Creatine pathway (Homo sapiens)

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ArcPathVisio Brace Ellipse EndoplasmicReticulum GolgiApparatus HexagonPathVisio MimDegradation Mitochondria Octagon PentagonPathVisio Rectangle RoundedRectangle SarcoplasmicReticulum TriangleEquilateralEast TrianglePathVisio none Creatine uptake:Mainly brain, muscleArginine/glycineamidinotransferasedeficiencyOrnithine aminotransferase deficiencyCreatine synthesis:Kidney, Liver, Pancreas, BrainGuanidinoacetate methyltransferase deficiencyUrinary creatinine excretionNon-enzymatic conversionCreatinetransporterdeficiencySLC6A8ArgininecreatinineCKS-adenosylmethionineOATGAMTVitamin B6CreatineGuanidinoacetateADPPyrroline-5-carboxylateCKADPCreatineGATMATPglutamateGlutamate-5-semialdehydeprolinePhosphocreatineATPORNITHINES-AdenosylhomocysteineGlycinecreatinine2-oxoglutaratePhospho-guanidinoacetateName: Creatine PahtwayOrganism: Homo sapiens


Description

Creatine pathway

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Quality Tags

Image:Featured.pngReview changes for Featured
Image:Curated.pngReview changes for Approved
Image:Wplogo_31.pngCommunity: Inborn Errors of Metabolism (IEM)
Image:Wplogo_31.pngCommunity: Rare Diseases

Ontology Terms

Bibliography

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  1. Atherton RS, Laws JF, Miles BJ, Thomson AR; ''Brain adenosine 5'-triphosphate-creatine phosphotransferase.''; Biochem J, 1970 PubMed Europe PMC Scholia
  2. Pemberton TA, Tanner JJ; ''Structural basis of substrate selectivity of Δ(1)-pyrroline-5-carboxylate dehydrogenase (ALDH4A1): semialdehyde chain length.''; Arch Biochem Biophys, 2013 PubMed Europe PMC Scholia
  3. Rossi JJ, Vender J, Berg CM, Coleman WH; ''Partial purification and some properties of delta1-pyrroline-5-carboxylate reductase from Escherichia coli.''; J Bacteriol, 1977 PubMed Europe PMC Scholia
  4. Sekhar PN, Amrutha RN, Sangam S, Verma DP, Kishor PB; ''Biochemical characterization, homology modeling and docking studies of ornithine delta-aminotransferase--an important enzyme in proline biosynthesis of plants.''; J Mol Graph Model, 2007 PubMed Europe PMC Scholia
  5. HOBSON GE, REES KR; ''The annelid phosphokinases.''; Biochem J, 1957 PubMed Europe PMC Scholia
  6. Komoto J, Huang Y, Takata Y, Yamada T, Konishi K, Ogawa H, Gomi T, Fujioka M, Takusagawa F; ''Crystal structure of guanidinoacetate methyltransferase from rat liver: a model structure of protein arginine methyltransferase.''; J Mol Biol, 2002 PubMed Europe PMC Scholia
  7. Shirai H, Blundell TL, Mizuguchi K; ''A novel superfamily of enzymes that catalyze the modification of guanidino groups.''; Trends Biochem Sci, 2001 PubMed Europe PMC Scholia

History

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CompareRevisionActionTimeUserComment
138099
Featured
Approved
view22:37, 14 March 2025EgonwAdded missing stereo, based on Rhea reactions.
128231
RARE
view13:39, 29 January 2024EweitzSoften disease color
128183view22:59, 28 January 2024EweitzFix label rendering issue in site by not PathVisio; refine alignments
122630view10:54, 22 April 2022FehrhartUpdated pathway node layout
122555view02:13, 16 April 2022EweitzModified title
122509
IEM
view12:31, 13 April 2022DeSladded chapter nr
122508view12:29, 13 April 2022DeSlLayout updates
122343view12:23, 21 March 2022TimZottiAdd corrected line connection
122308view16:26, 17 March 2022DeSlOntology Term : 'cerebral creatine deficiency syndrome' added !
122307view16:23, 17 March 2022DeSlOntology Term : 'Fanconi syndrome pathway' added !
122306view16:23, 17 March 2022DeSlOntology Term : 'Fanconi renotubular syndrome 1' added !
122305view16:21, 17 March 2022DeSlOntology Term : 'optic atrophy' added !
122304view16:21, 17 March 2022DeSlOntology Term : 'gyrate atrophy' added !
122303view16:20, 17 March 2022DeSlOntology Term : 'gyrate atrophy pathway' added !
122302view16:20, 17 March 2022DeSlOntology Term : 'guanidinoacetate methyltransferase deficiency pathway' added !
122301view16:19, 17 March 2022DeSlAdded PW DataNodes
122300view16:15, 17 March 2022DeSlUpdated RHEA-ID structure
122299view16:13, 17 March 2022DeSlUpdated ID for CK to reviewed UniProt ID
122298view16:11, 17 March 2022DeSlOntology Term : 'DOID:0060174' removed !
122297view16:07, 17 March 2022DeSllast layout change
122296view16:06, 17 March 2022DeSlMore layout updates, unified one OMIM ID to correct URL
122295view15:48, 17 March 2022DeSllayout of DataNodes, added alternative name for GATM
122294view15:46, 17 March 2022DeSlUpdated reaction directionality GATM protein
122259view13:40, 16 March 2022TimZottiAdded diseases and omim links
122256view13:32, 16 March 2022TimZottiAdded right metabolites
122231view10:31, 16 March 2022TimZottiOntology Term : 'disease pathway' added !
122230view10:30, 16 March 2022TimZottiOntology Term : 'classic metabolic pathway' added !
122225view10:11, 16 March 2022TimZottiAdded the correct omim links
122188view11:49, 15 March 2022TimZottiAdded disease for OAT
122187view11:34, 15 March 2022TimZottiAdded OMIM links

Connected the not connected lines Added literature references Added a legend

Added the correct CK protein
122150view11:45, 14 March 2022TimZottiOntology Term : 'GABA aminotransferase deficiency' added !
122149view11:44, 14 March 2022TimZottiOntology Term : 'guanidinoacetate methyltransferase deficiency' added !
122148view11:43, 14 March 2022TimZottiOntology Term : 'creatine transporter deficiency' added !
122147view11:43, 14 March 2022TimZottiOntology Term : 'AGAT deficiency' added !
122146view11:42, 14 March 2022TimZottiOntology Term : 'creatine metabolic pathway' added !
121597view18:06, 21 February 2022Andrafix typo
121550view10:36, 21 February 2022TimZottiModified description
121549view10:35, 21 February 2022TimZottiModified description
121543view10:11, 21 February 2022TimZottiI added the correct Rhea identifiers to the conversions
121535view09:57, 21 February 2022TimZottiChanged CK to protein instead of metabollite Added bidirectional conversions
121346view07:48, 16 February 2022TimZottiNew pathway

External references

DataNodes

View all...
Name  ↓Type  ↓Database reference  ↓Comment  ↓
2-oxoglutarateMetaboliteCHEBI:16810 (ChEBI)
ADPMetaboliteCHEBI:456216 (ChEBI)
ATPMetaboliteCHEBI:30616 (ChEBI)
ArginineMetaboliteCHEBI:32682 (ChEBI)
CKProteinEC 2.7.3.2 (Enzyme Nomenclature)
CreatineMetaboliteCHEBI:16919 (ChEBI)
GAMTGeneProductENSG00000130005 (Ensembl)
GATMGeneProductENSG00000171766 (Ensembl)
Glutamate-5 -semialdehydeMetaboliteQ2823261 (Wikidata)
GlycineMetaboliteHMDB00123 (HMDB)
GuanidinoacetateMetaboliteCHEBI:16344 (ChEBI)
OATGeneProductENSG00000065154 (Ensembl)
ORNITHINEMetaboliteCHEBI:45453 (ChEBI)
Phospho -guanidinoacetateMetaboliteCHEBI:16034 (ChEBI)
PhosphocreatineMetaboliteHMDB01511 (HMDB)
Pyrroline-5-carboxylateMetaboliteCHEBI:15893 (ChEBI)
S-AdenosylhomocysteineMetaboliteCHEBI:16680 (ChEBI)
S-adenosylmethionineMetaboliteCHEBI:15414 (ChEBI)
SLC6A8GeneProductENSG00000130821 (Ensembl)
Vitamin B6MetaboliteCHEBI:27306 (ChEBI)
creatinineMetaboliteCHEBI:16737 (ChEBI)
glutamateMetaboliteCHEBI:14321 (ChEBI)
prolineMetaboliteCHEBI:26271 (ChEBI)

Annotated Interactions

View all...
Source  ↓Target  ↓Type  ↓Database reference  ↓Comment  ↓
2-oxoglutarateGlutamate-5 -semialdehydemim-conversionRHEA: 25163 (Rhea) This conversion is bidirectional. I added two lines to make it more clear for the computer
2-oxoglutaratemim-conversionRhea: 25163 (Rhea) This conversion is bidirectional. I added two lines to make it more clear for the computer.
CreatinePhosphocreatinemim-conversionRHEA: 17160 (Rhea) This conversion is bidirectional. I added two lines to make it more clear for the computer
Glutamate-5 -semialdehydePyrroline-5-carboxylatemim-conversionRHEA:28237 (Rhea) This conversion is bidirectional. I added two lines to make it more clear for the computer
GuanidinoacetateCreatinemim-conversionRHEA: 10657 (Rhea)
GuanidinoacetatePhospho -guanidinoacetateRhea: 14148 (Rhea) This conversion is bidirectional. I added two lines to make it more clear for the computer.
GuanidinoacetatePhospho -guanidinoacetatemim-conversionRHEA:14148 (Rhea) This conversion is bidirectional. I added two lines to make it more clear for the computer.
Guanidinoacetatemim-conversionRHEA:13201 (Rhea)
ORNITHINEmim-conversionRHEA: 13201 (Rhea)
PhosphocreatineCreatinemim-conversionRhea: 17160 (Rhea) This conversion is bidirectional. I added two lines to make it more clear for the computer
Pyrroline-5-carboxylateGlutamate-5 -semialdehydemim-conversionRhea: 28237 (Rhea) This conversion is bidirectional. I added two lines to make it more clear for the computer
Pyrroline-5-carboxylateprolinemim-conversionRHEA:14112 (Rhea) This conversion is bidirectional. I added two lines to make it more clear for the computer
prolinePyrroline-5-carboxylatemim-conversionRhea:14112 (Rhea) This conversion is bidirectional. I added two lines to make it more clear for the computer
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