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. 1972 Oct;69(10):2807–2811. doi: 10.1073/pnas.69.10.2807

3-Hydroxypropionate: Significance of β-Oxidation of Propionate in Patients with Propionic Acidemia and Methylmalonic Acidemia

Toshiyuki Ando *, Karsten Rasmussen *, William L Nyhan *,‡, David Hull †
PMCID: PMC389649  PMID: 4507604

Abstract

[l-14C]Propionate administered intravenously was metabolized to methylmalonate, to 3-hydroxypropionate, and to methylcitrate in the urine of a patient with methylmalonic acidemia. L-[U-14C]Isoleucine and L-[U-14C]valine were also converted to urinary methylmalonate and to 3-hydroxypropionate in the patient. Two patients with propionic acidemia due to a defect in propionyl-CoA carboxylase metabolized [l-14C]propionate to uninary methylcitrate and 3-hydroxypropionate. The appearance of radioactive 3-hydroxypropionate in the urine after the administration of these compounds indicates that β-oxidation of propionyl-CoA through acryloyl-CoA was functioning in these patients. The conversion of valine to 3-hydroxypropionate suggests that valine is oxidized by way of propionate and propionyl-CoA in man.

Keywords: inborn errors, metabolic blocks, valine oxidation

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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