EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|---|
1.2.1.25 | mitochondrion | - |
Homo sapiens | 5739 | - |
EC Number | Organism | UniProt | Comment | Textmining |
---|---|---|---|---|
1.2.1.25 | Homo sapiens | P12694 AND P21953 | alpha and beta subunits of complex component E1 (BCKD), a tetramer (alpha2beta2), cf. EC 1.2.4.4 | - |
1.2.4.4 | Homo sapiens | A0A024R0K3 | subunit alpha | - |
2.3.1.168 | Homo sapiens | P09622 | cf. EC 1.8.1.4 | - |
EC Number | Source Tissue | Comment | Organism | Textmining |
---|---|---|---|---|
1.2.1.25 | astrocyte | - |
Homo sapiens | - |
1.2.1.25 | brain | 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 | Homo sapiens | - |
1.2.1.25 | cerebellum | 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 | Homo sapiens | - |
1.2.1.25 | cerebral cortex | - |
Homo sapiens | - |
1.2.1.25 | endothelium | - |
Homo sapiens | - |
1.2.1.25 | hippocampus | - |
Homo sapiens | - |
1.2.1.25 | 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 | Homo sapiens | - |
1.2.1.25 | neocortex | - |
Homo sapiens | - |
1.2.1.25 | neuron | - |
Homo sapiens | - |
1.2.4.4 | brain | predominantly in neuropil and neurons throughout the brain | Homo sapiens | - |
1.2.4.4 | neuron | - |
Homo sapiens | - |
1.2.4.4 | vascular endothelial cell | - |
Homo sapiens | - |
EC Number | Synonyms | Comment | Organism |
---|---|---|---|
1.2.1.25 | BCKD complex | - |
Homo sapiens |
1.2.1.25 | branched-chain alpha-ketoacid dehydrogenase complex | - |
Homo sapiens |
1.2.1.25 | branched-chain alpha-ketoacid dehydrogenase complex E1alpha subunit | - |
Homo sapiens |
1.2.1.25 | More | cf. EC 1.2.4.4 | Homo sapiens |
1.2.4.4 | 2-oxoisovalerate dehydrogenase subunit alpha | - |
Homo sapiens |
1.2.4.4 | BCKDHA | - |
Homo sapiens |
2.3.1.168 | DLD | - |
Homo sapiens |
EC Number | General Information | Comment | Organism |
---|---|---|---|
1.2.4.4 | physiological function | branched-chain amino acids are metabolized within both the vasculature and neurons in the human brain. Glutamate dehydrogenase isozymes, branched-chain aminotransferase and the branched-chain alpha-ketoacid dehydrogenase proteins may operate in conjunction with astrocytic glutamate transporters and glutamine synthetase to regulate the availability of glutamate | Homo sapiens |
2.3.1.168 | physiological function | branched-chain amino acids are metabolized within both the vasculature and neurons in the human brain. Glutamate dehydrogenase isozymes, branched-chain aminotransferase and the branched-chain alpha-ketoacid dehydrogenase proteins may operate in conjunction with astrocytic glutamate transporters and glutamine synthetase to regulate the availability of glutamate | Homo sapiens |