Essential fatty acids in clinically stable children with propionic acidaemia

T. Decsi, W. Sperl, B. Koletzko

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Disturbances of fatty acid metabolism with accumulation of odd-chain fatty acids have been reported in propionic acidaemia (PA). It is not known whether the synthesis of long-chain polyunsaturated fatty acids (LCPUFA) is also affected. In five clinically stable children with PA (median age 8 years, range 3.5-9.5 years; median percentage fibroblast propionyl-CoA carboxylase activity 0.8, range 0.8-1.5), we determined the fatty acid composition of plasma phospholipids, triglycerides and sterol esters and compared the results with those of is age-matched healthy controls. Odd-numbered fatty acids were found in all samples of PA patients but in controls median values were zero. Percentage contributions of substrate (linoleic acid, C(18:2)ω-6) and principal product (arachidonic acid, C(20:4)ω-6) of ω-6 LCPUFA synthesis did not differ between patients and controls. Similarly, there were no differences between both groups in the substrate (α-linolenic acid, C(18:3)ω-3) and principal product (docosahexaenoic acid, C(22:6)ω-3 of ω-3 LCPUFA formation. We conclude that disturbances of fatty acid metabolism in clinically stable children with PA do not affect LCPUFA synthesis.

Original languageEnglish
Pages (from-to)778-782
Number of pages5
JournalJournal of Inherited Metabolic Disease
Volume20
Issue number6
DOIs
Publication statusPublished - 1997

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Propionic Acidemia
Essential Fatty Acids
Unsaturated Fatty Acids
Fatty Acids
Methylmalonyl-CoA Decarboxylase
alpha-Linolenic Acid
Docosahexaenoic Acids
Sterols
Linoleic Acid
Arachidonic Acid
Phospholipids
Esters
Triglycerides
Fibroblasts

ASJC Scopus subject areas

  • Genetics(clinical)
  • Genetics
  • Endocrinology

Cite this

Essential fatty acids in clinically stable children with propionic acidaemia. / Decsi, T.; Sperl, W.; Koletzko, B.

In: Journal of Inherited Metabolic Disease, Vol. 20, No. 6, 1997, p. 778-782.

Research output: Contribution to journalArticle

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