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BRENDA support

Literature summary for 1.2.4.4 extracted from

  • Cole, J.T.; Sweatt, A.J.; Hutson, S.M.
    Expression of mitochondrial branched-chain aminotransferase and alpha-keto-acid dehydrogenase in rat brain: implications for neurotransmitter metabolism (2012), Front. Neuroanat., 6, 18.
    View publication on PubMedView publication on EuropePMC

Organism

Organism UniProt Comment Textmining
Rattus norvegicus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
cerebellum
-
Rattus norvegicus
-
cerebral cortex
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Rattus norvegicus
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hippocampus
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Rattus norvegicus
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hypothalamus
-
Rattus norvegicus
-
additional information not detected in astrocytes and Bergmann glia cells Rattus norvegicus
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neuron
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Rattus norvegicus
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spinal cord
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Rattus norvegicus
-

Synonyms

Synonyms Comment Organism
BCKDC
-
Rattus norvegicus
branched-chain alpha-ketoacid dehydrogenase enzyme complex
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Rattus norvegicus

General Information

General Information Comment Organism
malfunction maple syrup urine disease results from genetic enzyme defects, which lead to accumulation of toxic levels of branched chain amino acids and branched-chain alpha-keto acids that result in brain swelling Rattus norvegicus