Essential fatty acids in clinically stable children with propionic acidaemia

Journal of Inherited Metabolic Disease

Volumn 20, Issue 6, 1997, Pages 778-782

  Decsi, T ^a,b   Sperl, W ^c,d   Koletzko, B ^a  

^a UNIVERSITY OF MUNICH   (Germany)

^b UNIVERSITY OF PÉCS   (Hungary)

^c UNIVERSITY OF INNSBRUCK   (Austria)

^d Salzburger Landeskliniken   (Austria)

Author keywords

[No Author keywords available]

Indexed keywords

ARACHIDONIC ACID; DOCOSAHEXAENOIC ACID; ESSENTIAL FATTY ACID; LINOLEIC ACID; LONG CHAIN FATTY ACID; PHOSPHOLIPID; POLYUNSATURATED FATTY ACID; PROPIONYL COENZYME A CARBOXYLASE; STEROL; TRIACYLGLYCEROL;

ARTICLE; CHILD; CLINICAL ARTICLE; ENZYME ACTIVITY; FATTY ACID METABOLISM; FATTY ACID SYNTHESIS; FIBROBLAST; HUMAN; HUMAN CELL; LIPID COMPOSITION; PHOSPHOLIPID BLOOD LEVEL; PROPIONIC ACIDEMIA;

AMINO ACID METABOLISM, INBORN ERRORS; CHILD; CHILD, PRESCHOOL; FATTY ACIDS, ESSENTIAL; FATTY ACIDS, UNSATURATED; HUMANS; PHOSPHOLIPIDS; PROPIONIC ACIDS; STEROLS; TRIGLYCERIDES;

EID: 0030828132     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1005315717106     Document Type: Article

References (11)

1. Decsi T, Koletzko B (1994a) Polyunsaturated fatty acids in infant nutrition. Acta Paediatr Suppl 395: 31-37.
2. Decsi T, Koletzko B (1994b) Fatty acid composition of plasma lipid classes in healthy subjects from birth to young adulthood. Eur J Pediatr 153: 520-525.
3. Decsi T, Zaknun D, Zaknun J, Sperl W, Koletzko B (1995) Long-chain polyunsaturated fatty acids in children with severe protein-energy malnutrition with and without human immunodeficiency virus-1 infection. Am J Clin Nutr 62: 1283-1288.
4. Fenton WA, Rosenberg LE (1995) Disorders of propionate and methylmalonate metabolism. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds The Metabolic Bases of Inherited Disease, 7th edn., McGraw-Hill, New York, 1423-1449.
5. Giovannini M, Agostoni C, Biasucci G, et al (1996) Fatty acid metabolism in phenylketonuria. Eur J Pediatr 155: S132-S135.
6. Koletzko B, Abiodun PO, Laryea MD, Bremer HJ(1986) Fatty acid composition of plasma lipids in Nigerian children with protein-energy malnutrition. Eur J Pediatr 145: 109-115.
7. Lynen F, Hopper-Kessel I, Eggerer H (1962) Zur Biochemie der Fettäure. I. Die Fettsäuresynthese der Hefe und die Bildung enzymgebundener Acetessigsäure. Biochem Z 3: 327-332.
8. Suormala T, Wick H, Bonjour JP, Baumgartner ER (1985) Rapid differential diagnosis of carboxylase deficiencies and evaluation of biotin responsiveness in a single blood sample. Clin Chim Acta 145: 151-162.
9. Voss A, Reinhart M, Sankarappa S, Sprecher H (1991) The metabolism of 7,10,13,16,19-docosapentaenoic acid to 4,7,10,13,16,19-dodosahexaenoic acid in rat liver is independent of a Δ4-desaturase. J Biol Chem 266: 19995-20000.
10. Wendel U (1989) Abnormality of odd-numbered long-chain fatty acids in erythrocyte membrane lipids from patients with disorders of propionate metabolism. Pediatr Res 25: 147-150.
11. Wendel U, Eißler A, Sperl W, Schadewaldt P (1995) On the difference between urinary metabolite excretion and odd-numbered fatty acid production in propionic and methylmalonic acidaemias. J Inher Metab Dis 18: 584-591.