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

  1. Toshiyuki Ando*,
  2. Karsten Rasmussen*,
  3. William L. Nyhan*,‡, and
  4. David Hull†
  1. *Department of Pediatrics, University of California, San Diego, La Jolla, Calif. 92037
  2. †Hospital for Sick Children, Great Ormond Street, London, England

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.

Footnotes

  • ↵ ‡ Requests for reprints should be addressed to Dr. Nyhan, Dept. of Pediatrics, U.C.S.D., P.O. Box 109, La Jolla, Calif. 92037.

« Previous | Next Article »Table of Contents

This Article

  1. PNAS 1972-10 vol. 69 no. 10 2807-2811
  1. » Abstract
  2. Full Text (PDF)
About Our New Site Design >>
Link: Advanced Search

This Week's Issue

  1. July 22, 2008, 105 (29)
  1. Current Issue
  1. Alert me to new issues of PNAS

Most