In:
Acta Biochimica Polonica, Polskie Towarzystwo Biochemiczne (Polish Biochemical Society), Vol. 65, No. 2 ( 2018-07-08), p. 303-308
Abstract:
Deficiency of long-chain polyunsaturated fatty acids in phenylketonuria: a cross-sectional study Abstract The etiology of altered blood fatty acid (FA) profile in phenylketonuria (PKU) is understood only partially. We aimed to determine whether FAs deficiency is dependent on diet or metabolic disturbances. The study comprised 40 PKU patients (20 female, 20 male; aged 11 to 35 years; 12 children and 28 adults) and 40 healthy subjects (HS; 20 female, 20 male, aged 18 to 33 years). We assessed the profile of FAs (gas chromatography/mass spectrometry) and analyzed the 72-hour dietary recalls. The amount of C14:0, C16:0 and C16:1n-7, C18:1n-9 did not differ between the analyzed groups. The percentage of C18:0 was higher, while C20:3n-9, C18:2n-6, C20:2n-6, C20:4n-6, C22:4n-6, C22:5n-6 and C22:6n-3 was lower in PKU than in HS. However, C18:3n-6, C18:3n-3 and n-6/n-3 ratio were higher in PKU patients. The C20:4n-6/C20:3n-6 ratio (reaction catalyzed by Δ5-desaturase), the C22:5n-6/C22:4n-6 and the C22:6n-3/C22:5n-3 ratio (both reactions catalyzed by Δ6-desaturase) were significantly lower in PKU patients. The deficiency of long-chain polyunsaturated fatty acids in PKU patients may result not only from inadequate supply but also from metabolic disturbances.
Type of Medium:
Online Resource
ISSN:
1734-154X
,
0001-527X
DOI:
10.18388/abp.2018_2565
Language:
Unknown
Publisher:
Polskie Towarzystwo Biochemiczne (Polish Biochemical Society)
Publication Date:
2018
detail.hit.zdb_id:
2086022-5
Bookmarklink
