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Literature summary for 1.2.1.25 extracted from

  • Xing, G.; Ren, M.; Verma, A.
    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 .
    View publication on PubMedView publication on EuropePMC
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Cloned(Commentary)

Cloned (Comment) Organism
quantitative real-time PCR enzyme expression analysis Rattus norvegicus

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
 Rattus norvegicus 5739
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
3-methyl-2-oxobutanoate + CoA + NAD+ Rattus norvegicus
-
 2-methylpropanoyl-CoA + CO2 + NADH + H+
-
 ir
3-methyl-2-oxobutanoate + CoA + NAD+ Rattus norvegicus Sprague-Dawley
-
 2-methylpropanoyl-CoA + CO2 + NADH + H+
-
 ir
3-methyl-2-oxopentanoate + CoA + NAD+ Rattus norvegicus
-
 2-methylbutanoyl-CoA + CO2 + NADH + H+
-
 ir
3-methyl-2-oxopentanoate + CoA + NAD+ Rattus norvegicus Sprague-Dawley
-
 2-methylbutanoyl-CoA + CO2 + NADH + H+
-
 ir
4-methyl-2-oxopentanoate + CoA + NAD+ Rattus norvegicus
-
 3-methylbutanoyl-CoA + CO2 + NADH + H+
-
 ir
4-methyl-2-oxopentanoate + CoA + NAD+ Rattus norvegicus Sprague-Dawley
-
 3-methylbutanoyl-CoA + CO2 + NADH + H+
-
 ir

Organism

Organism UniProt Comment Textmining
Rattus norvegicus
-
-
-
Rattus norvegicus Sprague-Dawley
-
-
-

Posttranslational Modification

Posttranslational Modification Comment Organism
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

Source Tissue Comment Organism Textmining
astrocyte primary cortical astrocyte cultures Rattus norvegicus
-
brain
-
 Rattus norvegicus
-
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
-
neuron primary cortical neuronal cultures Rattus norvegicus
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3-methyl-2-oxobutanoate + CoA + NAD+
-
 Rattus norvegicus 2-methylpropanoyl-CoA + CO2 + NADH + H+
-
 ir
3-methyl-2-oxobutanoate + CoA + NAD+
-
 Rattus norvegicus Sprague-Dawley 2-methylpropanoyl-CoA + CO2 + NADH + H+
-
 ir
3-methyl-2-oxopentanoate + CoA + NAD+
-
 Rattus norvegicus 2-methylbutanoyl-CoA + CO2 + NADH + H+
-
 ir
3-methyl-2-oxopentanoate + CoA + NAD+
-
 Rattus norvegicus Sprague-Dawley 2-methylbutanoyl-CoA + CO2 + NADH + H+
-
 ir
4-methyl-2-oxopentanoate + CoA + NAD+
-
 Rattus norvegicus 3-methylbutanoyl-CoA + CO2 + NADH + H+
-
 ir
4-methyl-2-oxopentanoate + CoA + NAD+
-
 Rattus norvegicus Sprague-Dawley 3-methylbutanoyl-CoA + CO2 + NADH + H+
-
 ir

Synonyms

Synonyms Comment Organism
BCKD
-
 Rattus norvegicus
BCKDalpha
-
 Rattus norvegicus
BCKDbeta
-
 Rattus norvegicus
branched chain keto-acid dehydrogenase
-
 Rattus norvegicus
branched-chain-keto-acid dehydrogenase alpha
-
 Rattus norvegicus
branched-chain-keto-acid dehydrogenase beta
-
 Rattus norvegicus
mitochondrial branched-chain keto-acid dehydrogenase
-
 Rattus norvegicus

Cofactor

Cofactor Comment Organism Structure
CoA
-
 Rattus norvegicus
NAD+
-
 Rattus norvegicus

Expression

Organism Comment Expression
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
Rattus norvegicus transforming growth factor beta treatment (0.010 mg/ml for 48 h) significantly increases pBCKD protein expression in astrocytes up

General Information

General Information Comment Organism
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
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
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