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Literature summary for 2.3.1.168 extracted from
- Maple syrup urine disease: domain structure, mutations and exon skipping in the dihydrolipoyl transacylase (E2) component of the branched-chain alpha-keto acid dehydrogenase complex (1991), Mol. Biol. Med., 8, 49-63.
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| DNA sequence determination, overexpression of wild-type, lipol-free apo-enzyme, and mutants in Escherichia coli | Bos taurus |
| gene E2, DNA sequence determination and analysis | Homo sapiens |
| gene E2, mapping to chromosome 1p31 | Homo sapiens |
Protein Variants
| Protein Variants | Comment | Organism |
|---|---|---|
| F214C | natural splicing mutant from WG-34 cells, related to the maple syrup urine disease, phenotype of homo- and heterozygous mutations in humans | Homo sapiens |
| H391N | enzymatically inactive | Bos taurus |
| H391Q | enzymatically inactive | Bos taurus |
Molecular Weight [Da]
| Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|
| 52000 | - |
24 * 52000, SDS-PAGE | Bos taurus |
| 1116000 | - |
amino acid determination and sedimentation equilibrium analysis | Bos taurus |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| 2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine | Homo sapiens | - |
CoA + enzyme N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine | - |
? |  |
| 2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine | Bos taurus | multienzyme complex, scheme of the reaction steps | CoA + enzyme N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Bos taurus | - |
- |
- |
| Homo sapiens | P11182 | - |
- |
Purification (Commentary)
| Purification (Comment) | Organism |
|---|---|
| recombinant wild-type, apo-E2 enzyme, and mutants from Escherichia coli | Bos taurus |
Reaction
| Reaction | Comment | Organism | Reaction ID |
|---|---|---|---|
| 2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine = CoA + enzyme N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine | His391 is involved in the catalytic reaction | Bos taurus |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| liver | - |
Bos taurus | - |
| liver | - |
Homo sapiens | - |
Specific Activity [micromol/min/mg]
| Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
|---|---|---|---|
| additional information | - |
recombinant enzyme | Bos taurus |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| 2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine | - |
Bos taurus | CoA + enzyme N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine | - |
? | |
| 2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine | - |
Homo sapiens | CoA + enzyme N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine | - |
? | |
| 2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine | multienzyme complex, scheme of the reaction steps | Bos taurus | CoA + enzyme N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine | - |
? | |
| isovaleryl-CoA + dihydrolipoamide | recombinant enzyme, i.e. E2 domain, overexpressed in Escherichia coli | Bos taurus | CoA + S-isovaleryldihydrolipoamide | - |
r | |
Subunits
| Subunits | Comment | Organism |
|---|---|---|
| 24-mer | 24 * 52000, SDS-PAGE | Bos taurus |
| More | enzyme shows the E2 structure of 3 folded domains: lipoyl-bearing, E3-binding, and inner core, typical for all E2 protein of alpha-keto acid dehydrogenases | Bos taurus |
| More | assembly of 24 E2 subunits into a cubic structure, forming the core of the mammalian branched-chain alpha-keto acid dehydrogenase multienzyme complex | Bos taurus |
| More | assembly of 24 E2 subunits into a cubic structure, forming the core of the mammalian branched-chain alpha-keto acid dehydrogenase multienzyme complex | Homo sapiens |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| More | enzyme is the E2 component of the multienzyme complex branched-chain alpha-keto acid dehydrogenase, i.e. branched-chain 2-oxo acid dehydrogenase | Bos taurus |
| More | enzyme is the E2 component of the multienzyme complex branched-chain alpha-keto acid dehydrogenase, i.e. branched-chain 2-oxo acid dehydrogenase | Homo sapiens |
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Release 2023.1 (January 2023)
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