• DOI:10.1016/j.bbadis.2019.165586
Corpus ID: 207900059

Intracellular calcium mishandling leads to cardiac dysfunction and ventricular arrhythmias in a mouse model of propionic acidemia.

@article{Tamayo2019IntracellularCM,
  title={Intracellular calcium mishandling leads to cardiac dysfunction and ventricular arrhythmias in a mouse model of propionic acidemia.},
  author={M. Tamayo and A. Fulgencio-Covi{\'a}n and J. A. Navarro-Garc{\'i}a and A. Val-Blasco and G. Ruiz-Hurtado and M. Gil-Fern{\'a}ndez and L. Mart{\'i}n-Nunez and J. Lopez and L. Desviat and C. Delgado and E. Richard and M. Fern{\'a}ndez-Velasco},
  journal={Biochimica et biophysica acta. Molecular basis of disease},
  year={2019},
  pages={
          165586
        }
}

M. Tamayo, A. Fulgencio-Covián, +9 authors M. Fernández-Velasco

Published 2019

Medicine, Chemistry

Biochimica et biophysica acta. Molecular basis of disease

Propionic acidemia (PA) is a rare metabolic disease associated with mutations in genes encoding the α and β subunits of the enzyme propionyl-CoA carboxylase. The accumulation of toxic metabolites results in mitochondrial dysfunction, increased reactive oxygen species production and oxidative damage, which have been associated with the disease pathophysiology. Clinical symptoms are heterogeneous and include cardiac complications, mainly cardiac dysfunction and arrhythmias, which are recognized… CONTINUE READING

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