Propionic Acidemia

Metabolites

(R)-Methylmalonyl-CoA

Formula: C25H40N7O19P3S (867.131248)

CAS ID: 73173-92-9

(S)-3-Hydroxyisobutyrate

Formula: C4H8O3 (104.0473418)

CAS ID: 26543-05-5

(S)-Methylmalonate semialdehyde

Formula: C4H6O3 (102.0316926)

CAS ID: 99043-16-0

2-Methylbutanoyl-CoA

Formula: C26H44N7O17P3S (851.1727164)

CAS ID: 6712-02-3

2-Oxoglutarate

Formula: C5H6O5 (146.0215226)

CAS ID: 328-50-7

3-Methyl-2-oxobutanoic acid

Formula: C5H8O3 (116.0473418)

CAS ID: 759-05-7

4-Methyl-2-oxopentanoate

Formula: C6H10O3 (130.062991)

CAS ID: 816-66-0

Acetoacetate

Formula: C4H6O3 (102.0316926)

CAS ID: 541-50-4

Acetyl-CoA

Formula: C23H38N7O17P3S (809.1257688000001)

CAS ID: 72-89-9

ADP

Formula: C10H15N5O10P2 (427.02941500000003)

CAS ID: 58-64-0

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

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

Hydrogen peroxide

Formula: H2O2 (34.0054792)

CAS ID: 7722-84-1

L-3-Aminoisobutanoate

Formula: C4H9NO2 (103.0633254)

CAS ID: 4249-19-8

L-Glutamate

Formula: C5H9NO4 (147.0531554)

CAS ID: 56-86-0

L-Isoleucine

Formula: C6H13NO2 (131.0946238)

CAS ID: 73-32-5

L-Leucine

Formula: C6H13NO2 (131.0946238)

CAS ID: 61-90-5

L-Valine

Formula: C5H11NO2 (117.0789746)

CAS ID: 72-18-4

Lipoamide

Formula: C8H15NOS2 (205.05950199999998)

CAS ID: 940-69-2

Methylmalonate

Formula: C4H6O4 (118.0266076)

CAS ID: 516-05-2

NAD+

Formula: C21H28N7O14P2 (664.1169428000001)

CAS ID: 53-84-9

NADH

Formula: C21H29N7O14P2 (665.1247674)

CAS ID: 58-68-4

Oxygen

Formula: O2 (31.98983)

CAS ID: 7782-44-7

phosphate(3-)

Formula: O4P (94.953423)

CAS ID: 14265-44-2



Enzyme

EC Number name full name note
3.1.2.-
1.1.1.159 7alpha-hydroxysteroid dehydrogenase 7alpha-hydroxysteroid:NAD+ 7-oxidoreductase
1.1.1.178 3-hydroxy-2-methylbutyryl-CoA dehydrogenase (2S,3S)-3-hydroxy-2-methylbutanoyl-CoA:NAD+ oxidoreductase
1.1.1.239 3alpha(17beta)-hydroxysteroid dehydrogenase (NAD+) 3alpha(or 17beta)-hydroxysteroid:NAD+ oxidoreductase
1.1.1.35 3-hydroxyacyl-CoA dehydrogenase (S)-3-hydroxyacyl-CoA:NAD+ oxidoreductase
1.1.1.53 3alpha(or 20beta)-hydroxysteroid dehydrogenase 3alpha(or 20beta)-hydroxysteroid:NAD+ oxidoreductase
1.1.1.62 17beta-estradiol 17-dehydrogenase 17beta-estradiol:NAD(P)+ 17-oxidoreductase
1.2.1.3 aldehyde dehydrogenase (NAD+) aldehyde:NAD+ oxidoreductase
1.2.1.31 L-aminoadipate-semialdehyde dehydrogenase L-2-aminoadipate-6-semialdehyde:NAD(P)+ 6-oxidoreductase
1.2.1.8 betaine-aldehyde dehydrogenase betaine-aldehyde:NAD+ oxidoreductase
1.2.4.1 pyruvate dehydrogenase (acetyl-transferring) pyruvate:[dihydrolipoyllysine-residue acetyltransferase]-lipoyllysine 2-oxidoreductase (decarboxylating, acceptor-acetylating)
1.3.8.1 short-chain acyl-CoA dehydrogenase short-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
1.3.8.5 short-chain 2-methylacyl-CoA dehydrogenase short-chain 2-methylacyl-CoA:electron-transfer flavoprotein 2-oxidoreductase
1.3.8.7 medium-chain acyl-CoA dehydrogenase medium-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
1.8.1.9 thioredoxin-disulfide reductase thioredoxin:NADP+ oxidoreductase
2.3.1.16 acetyl-CoA C-acyltransferase acyl-CoA:acetyl-CoA C-acyltransferase
2.3.1.61 dihydrolipoyllysine-residue succinyltransferase succinyl-CoA:enzyme-N6-(dihydrolipoyl)lysine S-succinyltransferase
2.3.1.9 acetyl-CoA C-acetyltransferase acetyl-CoA:acetyl-CoA C-acetyltransferase
2.3.3.10 hydroxymethylglutaryl-CoA synthase acetyl-CoA:acetoacetyl-CoA C-acetyltransferase (thioester-hydrolysing, carboxymethyl-forming)
2.6.1.19 4-aminobutyrate-2-oxoglutarate transaminase 4-aminobutanoate:2-oxoglutarate aminotransferase
2.6.1.22 (S)-3-amino-2-methylpropionate transaminase (S)-3-amino-2-methylpropanoate:2-oxoglutarate aminotransferase
2.8.3.5 3-oxoacid CoA-transferase succinyl-CoA:3-oxo-acid CoA-transferase
3.1.2.1 acetyl-CoA hydrolase acetyl-CoA hydrolase
3.1.2.2 palmitoyl-CoA hydrolase palmitoyl-CoA hydrolase
4.1.3.4 hydroxymethylglutaryl-CoA lyase (S)-3-hydroxy-3-methylglutaryl-CoA acetoacetate-lyase (acetyl-CoA-forming)
4.2.1.17 enoyl-CoA hydratase (3S)-3-hydroxyacyl-CoA hydro-lyase
5.3.3.8 DELTA3-DELTA2-enoyl-CoA isomerase (3Z/3E)-alk-3-enoyl-CoA (2E)-isomerase
6.4.1.1 pyruvate carboxylase pyruvate:carbon-dioxide ligase (ADP-forming)
6.4.1.3 propionyl-CoA carboxylase propanoyl-CoA:carbon-dioxide ligase (ADP-forming)


Proteins

Protein ID name full name