Article
Fasting alleviates metabolic alterations in mice with propionyl-CoA carboxylase deficiency due to Pcca mutation.
Communications biology - 29 May 2024
He Wentao, Marchuk Hannah, Koeberl Dwight, Kasumov Takhar, Chen Xiaoxin, Zhang Guo-Fang
Abstract excerpt
Propionic acidemia (PA), resulting from Pcca or Pccb gene mutations, impairs propionyl-CoA metabolism and induces metabolic alterations. While speculation exists that fasting might exacerbate metabolic crises in PA patients by accelerating the breakdown of odd-chain fatty acids and amino acids into propionyl-CoA, direct evidence is lacking. Our investigation into the metabolic effects of fasting in Pcca-/-(A138T)...
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