Any feedback?
Literature summary for 1.2.4.4 extracted from
- Conformational stability and thermodynamic characterization of the lipoic acid bearing domain of human mitochondrial branched chain alpha-ketoacid dehydrogenase (2004), Protein Sci., 13, 2483-2492.
Localization
| Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|
| mitochondrion | - |
Homo sapiens | 5739 | - |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| additional information | Homo sapiens | enzyme-deficiency leads to inherited marple syrup urine disease MSUD | ? | - |
? |  |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
Renatured (Commentary)
| Renatured (Comment) | Organism |
|---|---|
| isothermal urea denaturation and refolding kinetics of lipoic acid-bearing domain, 2-step mechanism, overview | Homo sapiens |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| additional information | enzyme-deficiency leads to inherited marple syrup urine disease MSUD | Homo sapiens | ? | - |
? | |
| additional information | E1 and E2 components of the complex act in tandem, the lipoic acid-bearing domain plays an important role in substrate channeling in oxidative decarboxylation of the branched chain alpha-ketoacids within the dehydrogenase multienzyme complex | Homo sapiens | ? | - |
? | |
Subunits
| Subunits | Comment | Organism |
|---|---|---|
| 24mer | E2 component | Homo sapiens |
| More | E2 component is important for conformational stability of the branched chain alpha-ketoacid dehydrogenase multienzyme complex underlying the folding of the small beta-barrel of lipoic acid-bearing domain, overview, a reversible 2-step mechanism for interconversion of the enzyme from native to denatured state without stable or kinetic intermediates | Homo sapiens |
| tetramer | E1 component | Homo sapiens |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| BCKD | - |
Homo sapiens |
| branched chain alpha-ketoacid dehydrogenase complex | - |
Homo sapiens |
| More | lipoic acid bearing domain hbLBD is a domain of the E2 component of the branched chain alpha-ketoacid dehydrogenase complex BCKD | Homo sapiens |
Temperature Stability [°C]
| Temperature Stability Minimum [°C] | Temperature Stability Maximum [°C] | Comment | Organism |
|---|---|---|---|
| additional information | - |
thermal and cold denaturation kinetics of lipoic acid-bearing domain | Homo sapiens |
Use of this online version of BRENDA is free under the CC BY 4.0 license. See terms of use for full details.
Release 2023.1 (January 2023)
Legal
Curated at
Member of
