Cardiac Metabolic Pathways Affected in the Mouse Model of Barth Syndrome

  title={Cardiac Metabolic Pathways Affected in the Mouse Model of Barth Syndrome},
  author={Yan Huang and Corey Powers and Satish K. Madala and Kenneth D. Greis and Wendy D. Haffey and Jeffrey Allen Towbin and Enkhsaikhan Purevjav and Sabzali Javadov and Arnold W. Strauss and Zaza Khuchua and Cecilia Zazueta},
  booktitle={PloS one},
Cardiolipin (CL) is a mitochondrial phospholipid essential for electron transport chain (ETC) integrity. CL-deficiency in humans is caused by mutations in the tafazzin (Taz) gene and results in a multisystem pediatric disorder, Barth syndrome (BTHS). It has been reported that tafazzin deficiency destabilizes mitochondrial respiratory chain complexes and affects supercomplex assembly. The aim of this study was to investigate the impact of Taz-knockdown on the mitochondrial proteomic landscape… CONTINUE READING
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