1.2.1.25: branched-chain alpha-keto acid dehydrogenase system
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For detailed information about branched-chain alpha-keto acid dehydrogenase system, go to the full flat file.
Word Map on EC 1.2.1.25
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1.2.1.25
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maple
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syrup
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pdha1
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dihydrolipoamide
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alpha2beta2
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transacylase
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keto-acids
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bckdhb
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dihydrolipoyl
- 1.2.1.25
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maple
- syrup
- pdha1
- dihydrolipoamide
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alpha2beta2
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transacylase
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keto-acids
- bckdhb
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dihydrolipoyl
Reaction
Synonyms
2-oxoisovalerate dehydrogenase, alpha-ketoisovalerate dehydrogenase, BCKD, BCKD complex, BCKDalpha, BCKDbeta, BCKDC, BCKDH, BCKDHA, BCKDHB, bkdA, BKDH, branched chain alpha-keto acid dehydrogenase complex, branched chain keto-acid dehydrogenase, branched-chain alpha-keto acid dehydrogenase complex, branched-chain alpha-ketoacid dehydrogenase, branched-chain alpha-ketoacid dehydrogenase complex, branched-chain alpha-ketoacid dehydrogenase complex E1alpha subunit, branched-chain ketoacid dehydrogenase, branched-chain-keto-acid dehydrogenase alpha, branched-chain-keto-acid dehydrogenase beta, dehydrogenase, 2-oxoisovalerate (acylating), E1alpha subunit, E1beta subunit, mitochondrial branched-chain keto-acid dehydrogenase, More
ECTree
1.2.1.25 branched-chain alpha-keto acid dehydrogenase systemAdvanced search results
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results (Source Tissue
Source Tissue on EC 1.2.1.25 - branched-chain alpha-keto acid dehydrogenase system
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SOURCE TISSUE 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
SOURCE
within the cerebellum, there is punctate labelling in the processes of cells within the Purkinje cell layer, most likely to be Bergmann astrocytes. The Purkinje cells themselves are unlabelled (12/18 brains examined) or weakly immunopositive. Within the granule cell layer, rosette like structures (glomeruli) are immunopositive. Within the deep cerebellar nuclei, there is strong granular immunopositivity in small glial cells in contrast to the largely immunonegative neurons
additional information
distribution of BCKD-E1alpha in the human brain, immunohistochemic analysis, overview. Within the hippocampus, neuronal labelling is strongest within the cornu ammonis area 4 (CA4), and weaker in successive CA regions through to CA1. In the cerebral cortex, larger pyramidal cells are strongly immunopositive. Vasculature labelling is observed throughout the cerebral cortex with only weak astrocytic labelling (9/18 brains examined), in the vicinity of the hippocampus and within the white matter. The subependymal and the ependymal tissue are immunopositive
additional information
BCKDC activity is highest in liver but present in all tissues tested. In the liver, BCKDC activity is high, with moderate activity in the kidney and heart, and limited active enzyme in skeletal muscle
additional information
human tissue distribution of the BCKD complex: BCKD is highly found in kidney, liver, brain, and heart, more moderately in skeletal muscle, stomach, and colon. Subcutaneous adipose tissue and small intestine show the lowest BCKD complex activity
additional information
tissue-specific preference for BCAA oxidation relative to other fuels
additional information
within the hippocampus, GDH labelling is entirely astrocytic. There is also labelling of astrocytes in the neocortex and white matter, but labelling in the white matter is less dense than that in the cortex. The subpial feltwork of astrocytic processes is strongly immunopositive for GDH in all brain regions, not just in the cerebrum. Neurons are largely unlabelled (16/18 brains examined), apart from occasional pyramidal neurons in the vicinity of blood vessels. The labelling of perivascular astrocytic processes is evident throughout the cerebral cortex and cerebellum, in places, the labelling also involves the adjacent endothelium
additional information
tissue-specific preference for BCAA oxidation relative to other fuels. The greatest fraction of total BCAA oxidation occurs in skeletal muscle, liver, and brown fat
additional information
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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
additional information
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BCKDC activity is highest in liver but present in all tissues tested. In the liver, BCKDC activity is high, with moderate activity in the kidney and heart, and limited active enzyme in skeletal muscle
additional information
tissue-specific preference for BCAA oxidation relative to other fuels
additional information
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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
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