Literature summary for 1.2.1.25 extracted from

Hull, J.; Usmari Moraes, M.; Brookes, E.; Love, S.; Conway, M.
Distribution of the branched-chain alpha-ketoacid dehydrogenase complex E1alpha subunit and glutamate dehydrogenase in the human brain and their role in neuro-metabolism (2018), Neurochem. Int., 112, 49-58 .

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Localization

Localization	Comment	Organism	GeneOntology No.	Textmining
mitochondrion	-	Homo sapiens	5739	-

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	P12694 AND P21953	alpha and beta subunits of complex component E1 (BCKD), a tetramer (alpha2beta2), cf. EC 1.2.4.4	-

Source Tissue

Source Tissue	Comment	Organism	Textmining
astrocyte	-	Homo sapiens	-
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	-
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	-
cerebral cortex	-	Homo sapiens	-
endothelium	-	Homo sapiens	-
hippocampus	-	Homo sapiens	-
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	-
neocortex	-	Homo sapiens	-
neuron	-	Homo sapiens	-

Synonyms

Synonyms	Comment	Organism
BCKD complex	-	Homo sapiens
branched-chain alpha-ketoacid dehydrogenase complex	-	Homo sapiens
branched-chain alpha-ketoacid dehydrogenase complex E1alpha subunit	-	Homo sapiens
More	cf. EC 1.2.4.4	Homo sapiens

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Release 2023.1 (January 2023)