3-Hydroxyisobutyric Aciduria
Metabolites
Enzyme
| EC Number | name | full name | note |
|---|---|---|---|
| 3.1.2.- | |||
| 1.2.1.27 | methylmalonate-semialdehyde dehydrogenase (CoA-acylating) | 2-methyl-3-oxopropanoate:NAD+ 3-oxidoreductase (CoA-propanoylating) | |
| 1.2.4.4 | 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) | 3-methyl-2-oxobutanoate:[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]-lipoyllysine 2-oxidoreductase (decarboxylating, acceptor-2-methylpropanoylating) | |
| 1.3.8.1 | short-chain acyl-CoA dehydrogenase | short-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase | |
| 1.3.8.4 | isovaleryl-CoA dehydrogenase | 3-methylbutanoyl-CoA:electron-transfer flavoprotein 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 | |
| 1.8.1.4 | dihydrolipoyl dehydrogenase | protein-N6-(dihydrolipoyl)lysine:NAD+ oxidoreductase | |
| 2.3.1.16 | acetyl-CoA C-acyltransferase | acyl-CoA:acetyl-CoA C-acyltransferase | |
| 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 | |
| 2.6.1.42 | branched-chain-amino-acid transaminase | branched-chain-amino-acid: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 | |
| 3.1.2.4 | 3-hydroxyisobutyryl-CoA hydrolase | 3-hydroxy-2-methylpropanoyl-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 | |
| 4.2.1.18 | methylglutaconyl-CoA hydratase | (S)-3-hydroxy-3-methylglutaryl-CoA hydro-lyase (trans-3-methylglutaconyl-CoA-forming) | |
| 4.2.1.56 | itaconyl-CoA hydratase | citramalyl-CoA hydro-lyase (itaconyl-CoA-forming) | |
| 5.3.3.8 | DELTA3-DELTA2-enoyl-CoA isomerase | (3Z/3E)-alk-3-enoyl-CoA (2E)-isomerase | |
| 6.4.1.3 | propionyl-CoA carboxylase | propanoyl-CoA:carbon-dioxide ligase (ADP-forming) | |
| 6.4.1.4 | methylcrotonoyl-CoA carboxylase | 3-methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming) |
Pathway Source
| Organism | Taxonomy Group | Enzyme List | Source Ratio |
|---|---|---|---|
| Mus musculus | vertebrate_mammalian | 1.2.1.27 / 1.2.4.4 / 1.3.8.1 / 1.3.8.4 / 1.3.8.5 / 1.3.8.7 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.22 / 2.6.1.42 / 2.8.3.5 / 3.1.2.- / 3.1.2.1 / 3.1.2.2 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 4.2.1.18 / 4.2.1.56 / 5.3.3.8 / 6.4.1.3 / 6.4.1.4 | 100.00% |
| Homo sapiens | vertebrate_mammalian | 1.2.1.27 / 1.2.4.4 / 1.3.8.1 / 1.3.8.4 / 1.3.8.5 / 1.3.8.7 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.22 / 2.6.1.42 / 2.8.3.5 / 3.1.2.- / 3.1.2.1 / 3.1.2.2 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 4.2.1.18 / 4.2.1.56 / 5.3.3.8 / 6.4.1.3 / 6.4.1.4 | 100.00% |
| Rattus norvegicus | vertebrate_mammalian | 1.2.1.27 / 1.2.4.4 / 1.3.8.1 / 1.3.8.4 / 1.3.8.5 / 1.3.8.7 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.22 / 2.6.1.42 / 2.8.3.5 / 3.1.2.- / 3.1.2.1 / 3.1.2.2 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 4.2.1.18 / 4.2.1.56 / 5.3.3.8 / 6.4.1.3 / 6.4.1.4 | 100.00% |
| Knufia peltigerae | MAGs | 1.2.1.27 / 1.2.4.4 / 1.3.8.1 / 1.3.8.4 / 1.3.8.5 / 1.3.8.7 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.1 / 3.1.2.2 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 5.3.3.8 / 6.4.1.3 / 6.4.1.4 | 84.00% |
| Penicillium ucsense | MAGs | 1.2.1.27 / 1.2.4.4 / 1.3.8.4 / 1.3.8.5 / 1.3.8.7 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.- / 3.1.2.1 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 5.3.3.8 / 6.4.1.3 / 6.4.1.4 | 80.00% |
| Emydomyces testavorans | MAGs | 1.2.1.27 / 1.2.4.4 / 1.3.8.4 / 1.3.8.5 / 1.3.8.7 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.1 / 3.1.2.2 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 4.2.1.18 / 5.3.3.8 / 6.4.1.4 | 80.00% |
| Aspergillus fumigatus Af293 | MAGs | 1.2.1.27 / 1.2.4.4 / 1.3.8.4 / 1.3.8.5 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.- / 3.1.2.1 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 4.2.1.18 / 6.4.1.3 / 6.4.1.4 | 76.00% |
| Aspergillus niger CBS 513.88 | MAGs | 1.2.1.27 / 1.2.4.4 / 1.3.8.5 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.- / 3.1.2.1 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 5.3.3.8 / 6.4.1.3 / 6.4.1.4 | 72.00% |
| Exophiala dermatitidis | fungi | 1.2.1.27 / 1.2.4.4 / 1.3.8.5 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.1 / 3.1.2.2 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 5.3.3.8 / 6.4.1.3 / 6.4.1.4 | 72.00% |
| Rasamsonia emersonii CBS 393.64 | MAGs | 1.2.1.27 / 1.2.4.4 / 1.3.8.4 / 1.3.8.5 / 1.3.8.7 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.1 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 6.4.1.3 / 6.4.1.4 | 72.00% |
| Coccidioides posadasii C735 delta SOWgp | MAGs | 1.2.1.27 / 1.2.4.4 / 1.3.8.5 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.1 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 4.2.1.18 / 5.3.3.8 / 6.4.1.3 / 6.4.1.4 | 72.00% |
| Aspergillus leporis | MAGs | 1.2.1.27 / 1.2.4.4 / 1.3.8.5 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.1 / 3.1.2.2 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 6.4.1.3 / 6.4.1.4 | 68.00% |
| Penicillium crustosum | fungi | 1.2.1.27 / 1.2.4.4 / 1.3.8.5 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.- / 3.1.2.1 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 6.4.1.3 / 6.4.1.4 | 68.00% |
| Aspergillus chevalieri | fungi | 1.2.1.27 / 1.2.4.4 / 1.3.8.5 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.- / 3.1.2.1 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 6.4.1.3 / 6.4.1.4 | 68.00% |
| Aspergillus fumigatus | fungi | 1.2.1.27 / 1.2.4.4 / 1.3.8.5 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.1 / 3.1.2.2 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 6.4.1.3 / 6.4.1.4 | 68.00% |
| Paraburkholderia graminis | bacteria | 1.2.1.27 / 1.3.8.1 / 1.3.8.4 / 1.3.8.7 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.- / 3.1.2.2 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 4.2.1.18 / 6.4.1.4 | 68.00% |
| Aspergillus aculeatus ATCC 16872 | MAGs | 1.2.1.27 / 1.2.4.4 / 1.3.8.5 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.- / 3.1.2.1 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 6.4.1.3 / 6.4.1.4 | 68.00% |
| Shewanella frigidimarina NCIMB 400 | MAGs | 1.2.1.27 / 1.2.4.4 / 1.3.8.1 / 1.3.8.4 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.2 / 4.1.3.4 / 4.2.1.17 / 4.2.1.18 / 5.3.3.8 / 6.4.1.3 / 6.4.1.4 | 68.00% |
| Penicillium citrinum | fungi | 1.2.1.27 / 1.2.4.4 / 1.3.8.5 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.- / 3.1.2.1 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 6.4.1.3 / 6.4.1.4 | 68.00% |
| Exophiala sideris | MAGs | 1.2.1.27 / 1.2.4.4 / 1.3.8.5 / 1.8.1.4 / 2.3.1.16 / 2.3.1.9 / 2.3.3.10 / 2.6.1.19 / 2.6.1.42 / 2.8.3.5 / 3.1.2.1 / 3.1.2.2 / 3.1.2.4 / 4.1.3.4 / 4.2.1.17 / 5.3.3.8 / 6.4.1.4 | 68.00% |