Brain metabolism modulates neuronal excitability in a mouse model of pyruvate dehydrogenase deficiency.

ABSTRACT: Glucose is the ultimate substrate for most brain activities that use carbon, including synthesis of the neurotransmitters glutamate and γ-aminobutyric acid via mitochondrial tricarboxylic acid (TCA) cycle. Brain metabolism and neuronal excitability are thus interdependent. However, the principles that govern their relationship are not always intuitive because heritable defects of brain glucose metabolism are associated with the paradoxical coexistence, in the same individual, of episodic neuronal hyperexcitation (seizures) with reduced basal cerebral electrical activity. One such prototypic disorder is pyruvate dehydrogenase (PDH) deficiency (PDHD). PDH is central to metabolism because it steers most of the glucose-derived flux into the TCA cycle. To better understand the pathophysiology o

SUBMITTER: Jakkamsetti V 

PROVIDER: S-EPMC6637765 | biostudies-literature | 2019 Feb

REPOSITORIES: biostudies-literature