Fructose-1,6-diphosphatase Deficiency

Metabolites

(S)-Lactate

Formula: C3H6O3 (90.0316926)

CAS ID: 79-33-4

2-Oxoglutarate

Formula: C5H6O5 (146.0215226)

CAS ID: 328-50-7

2-Phospho-D-glycerate

Formula: C3H7O7P (185.9929402)

CAS ID: 3443-57-0

2,3-Bisphospho-D-glycerate

Formula: C3H8O10P2 (265.9592728)

CAS ID: 14438-19-8

ADP

Formula: C10H15N5O10P2 (427.02941500000003)

CAS ID: 58-64-0

alpha-D-Glucose

Formula: C6H12O6 (180.0633852)

CAS ID: 492-62-6

ATP

Formula: C10H16N5O13P3 (506.9957476)

CAS ID: 56-65-5

beta-D-Glucose

Formula: C6H12O6 (180.0633852)

CAS ID: 492-61-5

beta-D-Glucose 6-phosphate

Formula: C6H13O9P (260.0297178)

CAS ID: 15209-12-8

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

D-Fructose 6-phosphate

Formula: C6H13O9P (260.0297178)

CAS ID: 643-13-0

D-Glucose

Formula: C6H12O6 (180.0633852)

CAS ID: 50-99-7

D-Glucose 6-phosphate

Formula: C6H13O9P (260.0297178)

CAS ID: 56-73-5

GDP

Formula: C10H15N5O11P2 (443.02433)

CAS ID: 146-91-8

Glyceraldehyde 3-phosphate

Formula: C3H7O6P (169.9980252)

CAS ID: 591-59-3

Glycerate 3-phosphate

Formula: C3H7O7P (185.9929402)

CAS ID: 820-11-1

Glycerone phosphate

Formula: C3H7O6P (169.9980252)

CAS ID: 57-04-5

GTP

Formula: C10H16N5O14P3 (522.9906626)

CAS ID: 86-01-1

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

HCO3-

Formula: HCO3 (60.9925696)

CAS ID: 71-52-3

Malate

Formula: C4H6O5 (134.0215226)

CAS ID: 617-48-1

NAD+

Formula: C21H28N7O14P2 (664.1169428000001)

CAS ID: 53-84-9

NADH

Formula: C21H29N7O14P2 (665.1247674)

CAS ID: 58-68-4

Orthophosphate

Formula: H3PO4 (97.9768968)

CAS ID: 7664-38-2

Oxaloacetate

Formula: C4H4O5 (132.00587339999998)

CAS ID: 328-42-7

Phosphoenolpyruvate

Formula: C3H5O6P (167.98237600000002)

CAS ID: 138-08-9

Pyruvate

Formula: C3H4O3 (88.0160434)

CAS ID: 127-17-3

Fructose-1,6-diphosphate

Formula: C6H14O12P2 (339.9960504)

CAS ID: 34693-23-7

phosphate(3-)

Formula: O4P (94.953423)

CAS ID: 14265-44-2



Enzyme

EC Number name full name note
2.6.99.-
1.1.1.27 L-lactate dehydrogenase (S)-lactate:NAD+ oxidoreductase
1.1.1.37 malate dehydrogenase (S)-malate:NAD+ oxidoreductase
1.2.1.12 glyceraldehyde-3-phosphate dehydrogenase (phosphorylating) D-glyceraldehyde-3-phosphate:NAD+ oxidoreductase (phosphorylating)
2.7.1.1 hexokinase ATP:D-hexose 6-phosphotransferase
2.7.1.33 pantothenate kinase ATP:(R)-pantothenate 4'-phosphotransferase
3.1.3.11 fructose-bisphosphatase D-fructose-1,6-bisphosphate 1-phosphohydrolase
3.1.3.9 glucose-6-phosphatase D-glucose-6-phosphate phosphohydrolase
4.1.1.32 phosphoenolpyruvate carboxykinase (GTP) GTP:oxaloacetate carboxy-lyase (adding GTP; phosphoenolpyruvate-forming)
4.1.2.13 fructose-bisphosphate aldolase D-fructose-1,6-bisphosphate D-glyceraldehyde-3-phosphate-lyase (glycerone-phosphate-forming)
4.2.1.11 phosphopyruvate hydratase 2-phospho-D-glycerate hydro-lyase (phosphoenolpyruvate-forming)
4.2.3.3 methylglyoxal synthase glycerone-phosphate phosphate-lyase (methylglyoxal-forming)
5.1.3.3 Aldose 1-epimerase Aldose 1-epimerase
5.3.1.1 triose-phosphate isomerase D-glyceraldehyde-3-phosphate aldose-ketose-isomerase
5.3.1.9 glucose-6-phosphate isomerase alpha-D-glucose-6-phosphate aldose-ketose-isomerase (configuration-inverting)
5.4.2.11 phosphoglycerate mutase (2,3-diphosphoglycerate-dependent) D-phosphoglycerate 2,3-phosphomutase (2,3-diphosphoglycerate-dependent)
5.4.2.2 phosphoglucomutase (alpha-D-glucose-1,6-bisphosphate-dependent) alpha-D-glucose 1,6-phosphomutase
5.4.2.4 Bisphosphoglycerate mutase 3-Phospho-D-glycerate 1,2-phosphomutase
6.4.1.1 pyruvate carboxylase pyruvate:carbon-dioxide ligase (ADP-forming)


Proteins

Protein ID name full name