Contribution of aminoacid catabolism to propionate production in methylmalonic acidaemia

Lancet. 1989 Jun 10;1(8650):1298-9. doi: 10.1016/s0140-6736(89)92689-5.

Abstract

The rates of propionate production and aminoacid catabolism in 5 children with methylmalonic acidaemia were measured by use of stable isotope techniques. Total propionate production was 55-186 mumol/kg per h, to which the maximum contribution of protein catabolism was 10-35 mumol/kg per h (5-40%). These findings indicate important sources of propionate other than protein catabolism, which may account for the limited efficacy of dietary protein restriction in treatment of methylmalonic acidaemia.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acyl Coenzyme A / biosynthesis*
  • Adult
  • Amino Acid Metabolism, Inborn Errors / blood*
  • Amino Acids / metabolism*
  • Child
  • Humans
  • Isoleucine / metabolism
  • Kinetics
  • Leucine / metabolism
  • Malonates / blood*
  • Methionine / metabolism
  • Methylmalonic Acid / blood*
  • Phenylalanine / metabolism
  • Threonine / metabolism
  • Time Factors
  • Valine / metabolism

Substances

  • Acyl Coenzyme A
  • Amino Acids
  • Malonates
  • Isoleucine
  • Threonine
  • propionyl-coenzyme A
  • Phenylalanine
  • Methylmalonic Acid
  • Methionine
  • Leucine
  • Valine