Browsing by Author "Storm-Mathisen, Jon"

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Propionate enters GABAergic neurons, inhibits GABA transaminase, causes GABA accumulation and lethargy in a model of propionic acidemia

Morland, Cecilie; Frøland, Anne-Sofie; Nguyen, Mi Mong Tran; Storm-Mathisen, Jon; Gundersen, Vidar; Rise, Frode; Hassel, Bjørnar (2018)

Propionic acidemia is the accumulation of propionate in blood due to dysfunction of propionyl-CoA carboxylase. The condition causes lethargy and striatal degeneration with motor impairment in humans. How propionate exerts ...