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Literature summary extracted from
- Divergent induction of branched-chain aminotransferases and phosphorylation of branched chain keto-acid dehydrogenase is a potential mechanism coupling branched-chain keto-acid-mediated-astrocyte activation to branched-chain amino acid depletion-mediated cognitive deficit after traumatic brain injury (2018), J. Neurotrauma, 35, 2482-2494 .
Cloned(Commentary)
| EC Number | Cloned (Comment) | Organism |
|---|---|---|
| 1.2.1.25 | quantitative real-time PCR enzyme expression analysis | Rattus norvegicus |
Localization
| EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|---|
| 1.2.1.25 | mitochondrion | - |
Rattus norvegicus | 5739 | - |
Natural Substrates/ Products (Substrates)
| EC Number | Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 1.2.1.25 | 3-methyl-2-oxobutanoate + CoA + NAD+ | Rattus norvegicus | - |
2-methylpropanoyl-CoA + CO2 + NADH + H+ | - |
ir |  |
| 1.2.1.25 | 3-methyl-2-oxobutanoate + CoA + NAD+ | Rattus norvegicus Sprague-Dawley | - |
2-methylpropanoyl-CoA + CO2 + NADH + H+ | - |
ir | |
| 1.2.1.25 | 3-methyl-2-oxopentanoate + CoA + NAD+ | Rattus norvegicus | - |
2-methylbutanoyl-CoA + CO2 + NADH + H+ | - |
ir | |
| 1.2.1.25 | 3-methyl-2-oxopentanoate + CoA + NAD+ | Rattus norvegicus Sprague-Dawley | - |
2-methylbutanoyl-CoA + CO2 + NADH + H+ | - |
ir | |
| 1.2.1.25 | 4-methyl-2-oxopentanoate + CoA + NAD+ | Rattus norvegicus | - |
3-methylbutanoyl-CoA + CO2 + NADH + H+ | - |
ir | |
| 1.2.1.25 | 4-methyl-2-oxopentanoate + CoA + NAD+ | Rattus norvegicus Sprague-Dawley | - |
3-methylbutanoyl-CoA + CO2 + NADH + H+ | - |
ir | |
Organism
| EC Number | Organism | UniProt | Comment | Textmining |
|---|---|---|---|---|
| 1.2.1.25 | Rattus norvegicus | - |
- |
- |
| 1.2.1.25 | Rattus norvegicus Sprague-Dawley | - |
- |
- |
Posttranslational Modification
| EC Number | Posttranslational Modification | Comment | Organism |
|---|---|---|---|
| 1.2.1.25 | phosphoprotein | BCKD phosphorylation by BCKD kinase (BCKDK) inactivates BCKD and cause neurocognitive dysfunction, whereas dephosphorylation by specific phosphatase restores BCKD activity | Rattus norvegicus |
Source Tissue
| EC Number | Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|---|
| 1.2.1.25 | astrocyte | primary cortical astrocyte cultures | Rattus norvegicus | - |
| 1.2.1.25 | brain | - |
Rattus norvegicus | - |
| 1.2.1.25 | additional information | BCKD protein expression is higher in primarily cultured cortical neurons than in astrocytes, whereas pBCKD protein level is higher in astrocytes than in cortical neurons | Rattus norvegicus | - |
| 1.2.1.25 | neuron | primary cortical neuronal cultures | Rattus norvegicus | - |
Substrates and Products (Substrate)
| EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 1.2.1.25 | 3-methyl-2-oxobutanoate + CoA + NAD+ | - |
Rattus norvegicus | 2-methylpropanoyl-CoA + CO2 + NADH + H+ | - |
ir | |
| 1.2.1.25 | 3-methyl-2-oxobutanoate + CoA + NAD+ | - |
Rattus norvegicus Sprague-Dawley | 2-methylpropanoyl-CoA + CO2 + NADH + H+ | - |
ir | |
| 1.2.1.25 | 3-methyl-2-oxopentanoate + CoA + NAD+ | - |
Rattus norvegicus | 2-methylbutanoyl-CoA + CO2 + NADH + H+ | - |
ir | |
| 1.2.1.25 | 3-methyl-2-oxopentanoate + CoA + NAD+ | - |
Rattus norvegicus Sprague-Dawley | 2-methylbutanoyl-CoA + CO2 + NADH + H+ | - |
ir | |
| 1.2.1.25 | 4-methyl-2-oxopentanoate + CoA + NAD+ | - |
Rattus norvegicus | 3-methylbutanoyl-CoA + CO2 + NADH + H+ | - |
ir | |
| 1.2.1.25 | 4-methyl-2-oxopentanoate + CoA + NAD+ | - |
Rattus norvegicus Sprague-Dawley | 3-methylbutanoyl-CoA + CO2 + NADH + H+ | - |
ir | |
Synonyms
| EC Number | Synonyms | Comment | Organism |
|---|---|---|---|
| 1.2.1.25 | BCKD | - |
Rattus norvegicus |
| 1.2.1.25 | BCKDalpha | - |
Rattus norvegicus |
| 1.2.1.25 | BCKDbeta | - |
Rattus norvegicus |
| 1.2.1.25 | branched chain keto-acid dehydrogenase | - |
Rattus norvegicus |
| 1.2.1.25 | branched-chain-keto-acid dehydrogenase alpha | - |
Rattus norvegicus |
| 1.2.1.25 | branched-chain-keto-acid dehydrogenase beta | - |
Rattus norvegicus |
| 1.2.1.25 | mitochondrial branched-chain keto-acid dehydrogenase | - |
Rattus norvegicus |
Cofactor
| EC Number | Cofactor | Comment | Organism | Structure |
|---|---|---|---|---|
| 1.2.1.25 | CoA | - |
Rattus norvegicus | |
| 1.2.1.25 | NAD+ | - |
Rattus norvegicus | |
Expression
| EC Number | Organism | Comment | Expression |
|---|---|---|---|
| 1.2.1.25 | Rattus norvegicus | BCKD and BCKDK mRNA decrease significantly immediately after CCI-induced traumatic brain injury (TBI) in the rat. Brain BCKD mRNA levels decrease significantly in the craniotomy, contralateral CCI-induced TBI, and ipsilateral CCI at 4 and 24 h post-TBI, and the decrease remains significant in the contralateral CCI-induced TBI 3 days post-CCI. Phosphorylated BCKD proteins (pBCKD) increase significantly in the ipsilateral-CCI-induced TBI hemisphere. Glutamate treatment (0.025 mM for 24 h) significantly decreases pBCKD protein in neurons | down |
| 1.2.1.25 | Rattus norvegicus | transforming growth factor beta treatment (0.010 mg/ml for 48 h) significantly increases pBCKD protein expression in astrocytes | up |
General Information
| EC Number | General Information | Comment | Organism |
|---|---|---|---|
| 1.2.1.25 | malfunction | BCKD phosphorylation by BCKD kinase (BCKDK) inactivates BCKD and causes neurocognitive dysfunction, whereas dephosphorylation by specific phosphatase restores BCKD activity. Increased phosphorylated BCKD leads to increased BCKA accumulation causing BCKA-mediated astrocyte activation, cell death, and cognitive dysfunction as found in maple syrup urine disease | Rattus norvegicus |
| 1.2.1.25 | metabolism | divergent induction of branched-chain aminotransferases and phosphorylation of branched chain keto-acid dehydrogenase is a potential mechanism coupling branched-chain keto-acid-mediated-astrocyte activation to branched-chain amino acid depletion-mediated cognitive deficit after traumatic brain injury. Proposed model depicting the coordination of BCAA metabolism between the putative neuron: astrocyte and microglia postinjury, overview | Rattus norvegicus |
| 1.2.1.25 | physiological function | the BCKA dehydrogenase (BCKD) complex catalyzes the irreversible oxidative decarboxylation of BCKAs to produce branched-chain acyl-CoA intermediates, which then follow separate catabolic pathways. Branched-chain keto-acids (BCKAs) are metabolized by the mitochondrial branched-chain keto-acid dehydrogenase (BCKD) whose activity is regulated by its phosphorylation state. BCKD phosphorylation by BCKD kinase (BCKDK) inactivates BCKD and causes neurocognitive dysfunction, whereas dephosphorylation by specific phosphatase restores BCKD activity. Brain BCKD activity is dynamically regulated by the balance between its phosphorylation and dephosphorylation cycle | Rattus norvegicus |
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Release 2023.1 (January 2023)
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