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Urinary propionylcarnitine analysis for monitoring carnitine supplementation in inherited disorders of propionate metabolism

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Journal of Inherited Metabolic Disease

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References

  • Kidouchi K, Sugiyama N, Morishita H, Wada Y, Nagai S, Sakakibara J (1987) Analytical method for urinary glutarylcarnitine, acetylcarnitine and propionylcarnitine with a carboxylic acid analyser and a reversed-phase column.J Chromatogr 423: 297–303.

    Google Scholar 

  • McGarry JD, Foster DW (1976) An improved and simplified radioisotopic assay for the determination of free and esterified carnitine.J Lipid Res 17: 277–281.

    Google Scholar 

  • Roe CR, Millington DS, Maltby DA, Bohan TP (1984)l-Carnitine enhances excretion of propionyl coenzyme A as propionylcarnitine in propionic acidemia.J Clin Invest 73: 1785–1788.

    Google Scholar 

  • Sugiyama N, Morishita H, Nagaya S et al (1984) Biochemical evidence of carnitine effect on propionate elimination.J Inher Metab Dis 7: 137–138.

    Google Scholar 

  • Sugiyama N, Matsuda I, Wada Y et al (1989) Effect of levo-carnitine chloride (l-carnitine) on propionic acidemia, and vitamin B12 unresponsive and responsive methylmalonic acidemia in nineteen Japanese institutes. (II) Biochemical evaluation.J Jpn Pediatr Soc 93: 1818–1827 (abstract in English).

    Google Scholar 

  • Sugiyama N, Kidouchi K, Kobayashi M, Wada Y (1990) Carnitine deficiency in inherited organic acid disorders and Reye syndrome.Acta Paediatr Jpn 32: 410–416.

    Google Scholar 

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Authors and Affiliations

  1. Department of Pediatrics, Nagoya City University Medical School, Kawasumi, Mizuho-cho, Mizuho-ku, 467, Nagoya

    N. Sugiyama, Y. Wada & M. Kobayashi

  2. Department of Pediatrics, Kumamoto University Medical School, Japan

    I. Matsuda

  3. Department of Biochemical Genetics, Tohoku University School of Medicine, Japan

    K. Narisawa

  4. Department of Pediatrics, Medical Nagoya City Higashi General Hospital, Japan

    K. Kidouchi

Authors
  1. N. Sugiyama
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  2. I. Matsuda
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  3. Y. Wada
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  4. K. Narisawa
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  5. M. Kobayashi
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  6. K. Kidouchi
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Sugiyama, N., Matsuda, I., Wada, Y. et al. Urinary propionylcarnitine analysis for monitoring carnitine supplementation in inherited disorders of propionate metabolism. J Inherit Metab Dis 17, 611–615 (1994). https://doi.org/10.1007/BF00711600

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  • DOI: https://doi.org/10.1007/BF00711600

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