A zebrafish model of human Barth syndrome reveals the essential role of tafazzin in cardiac development and function.

@article{Khuchua2006AZM,
  title={A zebrafish model of human Barth syndrome reveals the essential role of tafazzin in cardiac development and function.},
  author={Zaza Khuchua and Zou Yue and Lorene Batts and Arnold Strauss},
  journal={Circulation research},
  year={2006},
  volume={99 2},
  pages={
          201-8
        }
}
Barth syndrome is an X-linked disorder characterized by cardiomyopathy, skeletal myopathy, neutropenia, organic aciduria, and growth retardation caused by mutations in tafazzin. The sequence similarity of tafazzin to acyltransferases suggests a role in mitochondrial phospholipid metabolism. To study the role of tafazzin in heart function and development, we created a knockdown zebrafish model. Zebrafish tafazzin mRNA is first evident at 7 hours post-fertilization (hpf). At 10 and 24 hpf… CONTINUE READING

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