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Odd-Numbered Long-Chain Fatty Acid Contents in Erythrocyte Membrane Phospholipids in Patients with an Impaired Propionate Utilization

  • U. Wendel
  • E. Diekmann
  • M. D. Laryea

Abstract

Patients with the inherited disorders of propionate and methylmalonate metabolism such as propionic acidaemia (PA; McKusick 23200) and methylmalonic acidurias (MMA; McKusick 25100) accumulate odd-numbered long-chain fatty acids (OLCFA) in lipids of various organs (Hommes et al., 1968; Gompertz, 1970; Gompertz et al., 1970; Kishimoto et al., 1973). The accumulation of OLCFA also occurs in cultured skin fibroblasts of PA and MMA patients (Giudici et al., 1986). This is due to the fact that intracellularly accumulated propionyl-CoA can replace acetyl-CoA as a ‘primer’ for long-chain fatty acid synthesis.

Keywords

Erythrocyte Membrane Culture Skin Fibroblast Methylmalonic Aciduria Propionic Acidemia Propionic Acidaemia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

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Copyright information

© Springer Science+Business Media Dordrecht 1988

Authors and Affiliations

  • U. Wendel
    • 1
  • E. Diekmann
    • 1
  • M. D. Laryea
    • 1
  1. 1.Metabolic UnitUniversity Children’s Hospital, DüsseldorfDüsseldorfFederal Republic of Germany

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