Metabolomics of dietary fatty acid restriction in patients with phenylketonuria.

ABSTRACT:

Background

Patients with phenylketonuria (PKU) have to follow a lifelong phenylalanine restricted diet. This type of diet markedly reduces the intake of saturated and unsaturated fatty acids especially long chain polyunsaturated fatty acids (LC-PUFA). Long-chain saturated fatty acids are substrates of mitochondrial fatty acid oxidation for acetyl-CoA production. LC-PUFA are discussed to affect inflammatory and haemostaseological processes in health and disease. The influence of the long term PKU diet on fatty acid metabolism with a special focus on platelet eicosanoid metabolism has been investigated in the study presented here.

Methodology/principal findings

12 children with PKU under good metabolic control and 8 healthy controls were included. Activated fatty acids (acylca

SUBMITTER: Mutze U 

PROVIDER: S-EPMC3418234 | biostudies-literature | 2012

REPOSITORIES: biostudies-literature