A novel disease with deficiency of mitochondrial very-long-chain acyl-CoA dehydrogenase.

@article{Aoyama1993AND,
  title={A novel disease with deficiency of mitochondrial very-long-chain acyl-CoA dehydrogenase.},
  author={Teruyoshi Aoyama and Yohzo Uchida and Richard I Kelley and Michael R Marble and Kurt Hofman and James H. Tonsgard and William James Rhead and Tadafumi Hashimoto},
  journal={Biochemical and biophysical research communications},
  year={1993},
  volume={191 3},
  pages={1369-72}
}
Palmitoyl-CoA dehydrogenase activity in skin fibroblasts from seven patients with unidentified defects of fatty acid oxidation was measured in the presence and absence of antibodies against medium-chain, long-chain, and very-long-chain acyl-CoA dehydrogenases (VLCAD). Two of the patients, 4-5 month old boys, were found to have a novel disease, VLCAD deficiency, as judged from the results of very low palmitoyl-CoA dehydrogenase activity and the lack of immunoreactivity toward antibody raised to… CONTINUE READING

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