Pyruvate Kinase Deficiency

Metabolites

(R)-Lactaldehyde

Formula: C3H6O2 (74.0367776)

CAS ID: 3946-09-6

(R)-Lactate

Formula: C3H6O3 (90.0316926)

CAS ID: 10326-41-7

(R)-S-Lactoylglutathione

Formula: C13H21N3O8S (379.10493060000005)

CAS ID: 25138-66-3

(S)-Lactaldehyde

Formula: C3H6O2 (74.0367776)

CAS ID: 3913-64-2

(S)-Lactate

Formula: C3H6O3 (90.0316926)

CAS ID: 79-33-4

(S)-Malate

Formula: C4H6O5 (134.0215226)

CAS ID: 97-67-6

Acetaldehyde

Formula: C2H4O (44.0262134)

CAS ID: 75-07-0

Acetate

Formula: C2H4O2 (60.0211284)

CAS ID: 64-19-7

Acetyl adenylate

Formula: C12H16N5O8P (389.0736466)

CAS ID: 13015-87-7

Acetyl phosphate

Formula: C2H5O5P (139.987461)

CAS ID: 590-54-5

Acetyl-CoA

Formula: C23H38N7O17P3S (809.1257688000001)

CAS ID: 72-89-9

ADP

Formula: C10H15N5O10P2 (427.02941500000003)

CAS ID: 58-64-0

AMP

Formula: C10H14N5O7P (347.06308240000004)

CAS ID: 61-19-8

ATP

Formula: C10H16N5O13P3 (506.9957476)

CAS ID: 56-65-5

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

Dihydrolipoamide

Formula: C8H17NOS2 (207.0751512)

CAS ID: 3884-47-7

GDP

Formula: C10H15N5O11P2 (443.02433)

CAS ID: 146-91-8

Glutathione

Formula: C10H17N3O6S (307.08380220000004)

CAS ID: 70-18-8

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

Lipoamide

Formula: C8H15NOS2 (205.05950199999998)

CAS ID: 940-69-2

Methylglyoxal

Formula: C3H4O2 (72.0211284)

CAS ID: 78-98-8

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

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

Propane-1,2-diol

Formula: C3H8O2 (76.0524268)

CAS ID: 57-55-6

Pyruvate

Formula: C3H4O3 (88.0160434)

CAS ID: 127-17-3

S-Acetyldihydrolipoamide-E

Formula: C10H19NO2S2 (249.0857154)

CAS ID:

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
2.7.11.-
1.1.1.21 aldose reductase alditol:NAD(P)+ 1-oxidoreductase
1.1.1.300 NADP-retinol dehydrogenase retinol:NADP+ oxidoreductase
1.1.1.372 D/L-glyceraldehyde reductase glycerol:NADP+ oxidoreductase (D/L-glyceraldehyde-forming)
1.1.1.40 malate dehydrogenase (oxaloacetate-decarboxylating) (NADP+) (S)-malate:NADP+ oxidoreductase (oxaloacetate-decarboxylating)
1.1.1.54 allyl-alcohol dehydrogenase allyl-alcohol:NADP+ oxidoreductase
1.1.99.39 D-2-hydroxyglutarate dehydrogenase (R)-2-hydroxyglutarate:acceptor 2-oxidoreductase
1.2.4.1 pyruvate dehydrogenase (acetyl-transferring) pyruvate:[dihydrolipoyllysine-residue acetyltransferase]-lipoyllysine 2-oxidoreductase (decarboxylating, acceptor-acetylating)
1.8.1.4 dihydrolipoyl dehydrogenase protein-N6-(dihydrolipoyl)lysine:NAD+ oxidoreductase
2.3.1.9 acetyl-CoA C-acetyltransferase acetyl-CoA:acetyl-CoA C-acetyltransferase
3.1.2.1 acetyl-CoA hydrolase acetyl-CoA hydrolase
3.6.1.7 acylphosphatase acylphosphate phosphohydrolase
4.1.1.32 phosphoenolpyruvate carboxykinase (GTP) GTP:oxaloacetate carboxy-lyase (adding GTP; phosphoenolpyruvate-forming)
4.4.1.5 lactoylglutathione lyase (R)-S-lactoylglutathione methylglyoxal-lyase (isomerizing; glutathione-forming)


Pathway Source

Organism Taxonomy Group Enzyme List Source Ratio
Mus musculus vertebrate_mammalian 1.1.1.21 / 1.1.1.300 / 1.1.1.372 / 1.1.1.40 / 1.1.1.54 / 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.1.1.32 / 4.4.1.5 100.00%
Rattus norvegicus vertebrate_mammalian 1.1.1.21 / 1.1.1.300 / 1.1.1.372 / 1.1.1.40 / 1.1.1.54 / 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.1.1.32 / 4.4.1.5 100.00%
Homo sapiens vertebrate_mammalian 1.1.1.21 / 1.1.1.300 / 1.1.1.372 / 1.1.1.40 / 1.1.1.54 / 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.1.1.32 / 4.4.1.5 100.00%
Cupriavidus taiwanensis bacteria 1.1.1.21 / 1.1.1.40 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.6.1.7 / 4.1.1.32 / 4.4.1.5 64.29%
Penicillium ucsense MAGs 1.1.1.372 / 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.4.1.5 64.29%
Polaromonas naphthalenivorans CJ2 MAGs 1.1.1.40 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.1.1.32 / 4.4.1.5 64.29%
Burkholderia vietnamiensis G4 MAGs 1.1.1.40 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.1.1.32 / 4.4.1.5 64.29%
Aspergillus niger fungi 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.4.1.5 57.14%
Aspergillus parasiticus MAGs 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.4.1.5 57.14%
Penicillium hordei fungi 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.4.1.5 57.14%
Exophiala dermatitidis fungi 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.4.1.5 57.14%
Oryza sativa Japonica Group MAGs 1.1.1.21 / 1.1.1.40 / 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.7.11.- / 3.6.1.7 / 4.4.1.5 57.14%
Aspergillus candidus fungi 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.4.1.5 57.14%
Penicillium crustosum fungi 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.4.1.5 57.14%
Aspergillus leporis MAGs 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.4.1.5 57.14%
Penicillium digitatum fungi 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.4.1.5 57.14%
Talaromyces islandicus MAGs 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.4.1.5 57.14%
Penicillium citrinum fungi 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.4.1.5 57.14%
Penicillium freii MAGs 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.4.1.5 57.14%
Penicillium solitum fungi 1.1.99.39 / 1.2.4.1 / 1.8.1.4 / 2.3.1.9 / 2.7.11.- / 3.1.2.1 / 3.6.1.7 / 4.4.1.5 57.14%