Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)

Metabolites

1-Pyrroline-2-carboxylate

Formula: C5H7NO2 (113.0476762)

CAS ID: 2139-03-9

2-Oxoglutarate

Formula: C5H6O5 (146.0215226)

CAS ID: 328-50-7

4-Hydroxy-L-glutamate

Formula: C5H9NO5 (163.0480704)

CAS ID: 533-62-0

ADP

Formula: C10H15N5O10P2 (427.02941500000003)

CAS ID: 58-64-0

Ammonia

Formula: NH3 (17.0265478)

CAS ID: 7664-41-7

AMP

Formula: C10H14N5O7P (347.06308240000004)

CAS ID: 61-19-8

ATP

Formula: C10H16N5O13P3 (506.9957476)

CAS ID: 56-65-5

Carbamoyl phosphate

Formula: CH4NO5P (140.9827104)

CAS ID: 590-55-6

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

Creatine

Formula: C4H9N3O2 (131.06947340000002)

CAS ID: 57-00-1

D-Proline

Formula: C5H9NO2 (115.0633254)

CAS ID: 344-25-2

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

Hydrogen peroxide

Formula: H2O2 (34.0054792)

CAS ID: 7722-84-1

Hydroxyproline

Formula: C5H9NO3 (131.0582404)

CAS ID: 51-35-4

L-Glutamate

Formula: C5H9NO4 (147.0531554)

CAS ID: 56-86-0

L-Glutamate 5-semialdehyde

Formula: C5H9NO3 (131.0582404)

CAS ID: 496-92-4

L-Ornithine

Formula: C5H12N2O2 (132.0898732)

CAS ID: 70-26-8

L-Proline

Formula: C5H9NO2 (115.0633254)

CAS ID: 147-85-3

NAD+

Formula: C21H28N7O14P2 (664.1169428000001)

CAS ID: 53-84-9

NADH

Formula: C21H29N7O14P2 (665.1247674)

CAS ID: 58-68-4

NADP+

Formula: C21H29N7O17P3 (744.0832754)

CAS ID: 53-59-8

Nitric oxide

Formula: NO (29.997989)

CAS ID: 10102-43-9

Orthophosphate

Formula: H3PO4 (97.9768968)

CAS ID: 7664-38-2

Oxaloacetate

Formula: C4H4O5 (132.00587339999998)

CAS ID: 328-42-7

Oxygen

Formula: O2 (31.98983)

CAS ID: 7782-44-7

Phosphocreatine

Formula: C4H10N3O5P (211.035806)

CAS ID: 67-07-2

Urea

Formula: CH4N2O (60.0323614)

CAS ID: 57-13-6

diphosphate

Formula: O7P2 (173.911931)

CAS ID: 14000-31-8

phosphate(3-)

Formula: O4P (94.953423)

CAS ID: 14265-44-2



Enzyme

EC Number name full name note
1.14.11.2 procollagen-proline 4-dioxygenase procollagen-L-proline,2-oxoglutarate:oxygen oxidoreductase (4-hydroxylating)
1.14.13.39 nitric-oxide synthase (NADPH) L-arginine,NADPH:oxygen oxidoreductase (nitric-oxide-forming)
1.2.1.88 L-glutamate gamma-semialdehyde dehydrogenase L-glutamate gamma-semialdehyde:NAD+ oxidoreductase
1.4.1.3 glutamate dehydrogenase [NAD(P)+] L-glutamate:NAD(P)+ oxidoreductase (deaminating)
1.4.3.3 D-amino-acid oxidase D-amino-acid:oxygen oxidoreductase (deaminating)
1.5.1.2 pyrroline-5-carboxylate reductase L-proline:NAD(P)+ 5-oxidoreductase
1.5.5.2 proline dehydrogenase L-proline:quinone oxidoreductase
2.1.1.2 guanidinoacetate N-methyltransferase S-adenosyl-L-methionine:N-guanidinoacetate methyltransferase
2.1.3.3 ornithine carbamoyltransferase carbamoyl-phosphate:L-ornithine carbamoyltransferase
2.1.4.1 glycine amidinotransferase L-arginine:glycine amidinotransferase
2.6.1.1 aspartate transaminase L-aspartate:2-oxoglutarate aminotransferase
2.6.1.13 ornithine aminotransferase L-ornithine:2-oxo-acid aminotransferase
2.6.1.3 cysteine transaminase L-cysteine:2-oxoglutarate aminotransferase
2.7.3.2 creatine kinase ATP:creatine N-phosphotransferase
3.5.3.1 arginase L-arginine amidinohydrolase
4.3.2.1 argininosuccinate lyase 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
6.1.1.15 proline-tRNA ligase L-proline:tRNAPro ligase (AMP-forming)
6.1.1.17 glutamate-tRNA ligase L-glutamate:tRNAGlu ligase (AMP-forming)
6.1.1.19 arginine-tRNA ligase L-arginine:tRNAArg ligase (AMP-forming)
6.3.4.16 carbamoyl-phosphate synthase (ammonia) carbon-dioxide:ammonia ligase (ADP-forming, carbamate-phosphorylating)
6.3.4.5 argininosuccinate synthase L-citrulline:L-aspartate ligase (AMP-forming)


Proteins

Protein ID name full name