Age-related accumulation of phytanic acid in plasma from patients with the cerebro-hepato-renal (Zellweger) syndrome

R. J. Wanders; W. Smit; H. S. Heymans; R. B. Schutgens; P. G. Barth; H. Schierbeek; G. P. Smit; R. Berger; H. Przyrembel; T. A. Eggelte

doi:10.1016/0009-8981(87)90193-8

Age-related accumulation of phytanic acid in plasma from patients with the cerebro-hepato-renal (Zellweger) syndrome

R. J. Wanders
, W. Smit
, H. S. Heymans
, R. B. Schutgens
, P. G. Barth
, H. Schierbeek
, G. P. Smit
, R. Berger
, H. Przyrembel
, T. A. Eggelte

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

Plasma samples from several Zellweger patients were found to contain elevated phytanic acid levels. It was subsequently found that the level of phytanic acid in plasma from Zellweger patients depends upon the age of the patients at the time of sampling. In patients 17 weeks of age or younger, plasma phytanic acid levels were found to be normal, whereas in patients 40 weeks of age or older plasma phytanic acid levels were found to be elevated. The relationship between the age of the patients at sampling and the level of phytanic acid in the patients' plasma is probably the resultant of dietary intake of phytanic acid combined with a defective catabolism of this compound

Original language	English
Pages (from-to)	45-56
Journal	Clinica chimica acta; international journal of clinical chemistry
Volume	166
Issue number	1
DOIs	https://doi.org/10.1016/0009-8981(87)90193-8
Publication status	Published - 1987

Access to Document

10.1016/0009-8981(87)90193-8

Cite this

Wanders, R. J., Smit, W., Heymans, H. S., Schutgens, R. B., Barth, P. G., Schierbeek, H., Smit, G. P., Berger, R., Przyrembel, H., & Eggelte, T. A. (1987). Age-related accumulation of phytanic acid in plasma from patients with the cerebro-hepato-renal (Zellweger) syndrome. Clinica chimica acta; international journal of clinical chemistry, 166(1), 45-56. https://doi.org/10.1016/0009-8981(87)90193-8

@article{8dc85edbe07f4caa933206b8629dd61e,

title = "Age-related accumulation of phytanic acid in plasma from patients with the cerebro-hepato-renal (Zellweger) syndrome",

abstract = "Plasma samples from several Zellweger patients were found to contain elevated phytanic acid levels. It was subsequently found that the level of phytanic acid in plasma from Zellweger patients depends upon the age of the patients at the time of sampling. In patients 17 weeks of age or younger, plasma phytanic acid levels were found to be normal, whereas in patients 40 weeks of age or older plasma phytanic acid levels were found to be elevated. The relationship between the age of the patients at sampling and the level of phytanic acid in the patients' plasma is probably the resultant of dietary intake of phytanic acid combined with a defective catabolism of this compound",

author = "Wanders, \{R. J.\} and W. Smit and Heymans, \{H. S.\} and Schutgens, \{R. B.\} and Barth, \{P. G.\} and H. Schierbeek and Smit, \{G. P.\} and R. Berger and H. Przyrembel and Eggelte, \{T. A.\}",

year = "1987",

doi = "10.1016/0009-8981(87)90193-8",

language = "English",

volume = "166",

pages = "45--56",

journal = "Clinica chimica acta; international journal of clinical chemistry",

issn = "0009-8981",

publisher = "Elsevier",

number = "1",

}

Wanders, RJ, Smit, W, Heymans, HS, Schutgens, RB, Barth, PG, Schierbeek, H, Smit, GP, Berger, R, Przyrembel, H & Eggelte, TA 1987, 'Age-related accumulation of phytanic acid in plasma from patients with the cerebro-hepato-renal (Zellweger) syndrome', Clinica chimica acta; international journal of clinical chemistry, vol. 166, no. 1, pp. 45-56. https://doi.org/10.1016/0009-8981(87)90193-8

TY - JOUR

T1 - Age-related accumulation of phytanic acid in plasma from patients with the cerebro-hepato-renal (Zellweger) syndrome

AU - Wanders, R. J.

AU - Smit, W.

AU - Heymans, H. S.

AU - Schutgens, R. B.

AU - Barth, P. G.

AU - Schierbeek, H.

AU - Smit, G. P.

AU - Berger, R.

AU - Przyrembel, H.

AU - Eggelte, T. A.

PY - 1987

Y1 - 1987

N2 - Plasma samples from several Zellweger patients were found to contain elevated phytanic acid levels. It was subsequently found that the level of phytanic acid in plasma from Zellweger patients depends upon the age of the patients at the time of sampling. In patients 17 weeks of age or younger, plasma phytanic acid levels were found to be normal, whereas in patients 40 weeks of age or older plasma phytanic acid levels were found to be elevated. The relationship between the age of the patients at sampling and the level of phytanic acid in the patients' plasma is probably the resultant of dietary intake of phytanic acid combined with a defective catabolism of this compound

AB - Plasma samples from several Zellweger patients were found to contain elevated phytanic acid levels. It was subsequently found that the level of phytanic acid in plasma from Zellweger patients depends upon the age of the patients at the time of sampling. In patients 17 weeks of age or younger, plasma phytanic acid levels were found to be normal, whereas in patients 40 weeks of age or older plasma phytanic acid levels were found to be elevated. The relationship between the age of the patients at sampling and the level of phytanic acid in the patients' plasma is probably the resultant of dietary intake of phytanic acid combined with a defective catabolism of this compound

U2 - 10.1016/0009-8981(87)90193-8

DO - 10.1016/0009-8981(87)90193-8

M3 - Article

C2 - 2440622

SN - 0009-8981

VL - 166

SP - 45

EP - 56

JO - Clinica chimica acta; international journal of clinical chemistry

JF - Clinica chimica acta; international journal of clinical chemistry

IS - 1

ER -

Age-related accumulation of phytanic acid in plasma from patients with the cerebro-hepato-renal (Zellweger) syndrome

Abstract

Access to Document

Fingerprint

Cite this