|
Journal of Lipid Research, Vol. 41, 1543-1551, October 2000
Copyright © 2000 by Lipid Research, Inc.
Urinary -tocopherol metabolites in -tocopherol transfer protein-deficient patients
Markus Schuelkea,
Angelika Elsnerb,
Barbara Finckhd,
Alfried Kohlschütterd,
Christoph Hübnera, and
Regina Brigelius-Flohéb,c
a Department of Neuropediatrics, Charité University Hospital, Humboldt University Berlin, D-13353 Berlin, Germany
b German Institute of Human Nutrition, D-14558 Potsdam-Rehbrücke, Germany
c Institute for Nutritional Science, University of Potsdam, D-14458 Potsdam-Rehbrücke, Germany
d Children's Hospital, University of Hamburg, D-20246 Hamburg, Germany
Correspondence to:
Regina Brigelius-Flohé
Patients with -tocopherol transfer protein ( -TTP) defects experience neurological symptoms characteristic of vitamin E deficiency and depend on continuous high -tocopherol supplements. We investigated the excretion of 2,5,7,8-tetramethyl-2(2'-carboxyethyl)-6-hydroxychroman ( -CEHC), a urinary metabolite of -tocopherol, as a putative marker for the -tocopherol status of -TTP-deficient patients and control subjects. In three patients vitamin E supplementation was stopped for short periods of time, during which plasma -tocopherol concentrations and urinary -CEHC excretion were measured. In the patients, plasma -tocopherol decreased below normal (<5 µmol/l) but -CEHC excretion remained above the range of unsupplemented control subjects (0.118;0.306 mg/day, n = 6). In healthy subjects, however, -CEHC excretion was increased only after surpassing a plasma -tocopherol threshold of 30;40 µmol/l. Such a threshold did not exist in patients. The general mechanism of -tocopherol degradation did not appear to differ between patients and control subjects.
The presumed mechanism of - and subsequent ß-oxidation was supported by the detection of -CPHC, an -CEHC homolog with a side chain longer by 3 carbon atoms, both in supplemented patients and in control subjects. Schuelke, M., A. Elsner, B. Finckh, A. Kohlschütter, C. Hübner, and R. Brigelius-Flohé. Urinary -tocopherol metabolites in -tocopherol transfer protein-deficient patients. J. Lipid Res. 2000. 41: 1543;1551.
Supplementary key words:
-tocopherol, -tocopherol metabolites, -CEHC, -tocopherol transfer protein, AVED patients

CiteULike Complore Connotea Del.icio.us Digg Reddit Technorati What's this?
This article has been cited by other articles:

|
 |

|
 |
 
A. J Clifford, F. F de Moura, C. C Ho, J. C Chuang, J. Follett, J. G Fadel, and J. A Novotny
A feasibility study quantifying in vivo human {alpha}-tocopherol metabolism
Am. J. Clinical Nutrition,
December 1, 2006;
84(6):
1430 - 1441.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. S Bruno, S. W Leonard, J. Li, T. M Bray, and M. G Traber
Lower plasma {alpha}-carboxyethyl-hydroxychroman after deuterium-labeled {alpha}-tocopherol supplementation suggests decreased vitamin E metabolism in smokers
Am. J. Clinical Nutrition,
May 1, 2005;
81(5):
1052 - 1059.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
S. W. Leonard, E. Gumpricht, M. W. Devereaux, R. J. Sokol, and M. G. Traber
Quantitation of rat liver vitamin E metabolites by LC-MS during high-dose vitamin E administration
J. Lipid Res.,
May 1, 2005;
46(5):
1068 - 1075.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
D. J. Harrington, R. Soper, C. Edwards, G. F. Savidge, S. J. Hodges, and M. J. Shearer
Determination of the urinary aglycone metabolites of vitamin K by HPLC with redox-mode electrochemical detection
J. Lipid Res.,
May 1, 2005;
46(5):
1053 - 1060.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. S Bruno, R. Ramakrishnan, T. J Montine, T. M Bray, and M. G Traber
{alpha}-Tocopherol disappearance is faster in cigarette smokers and is inversely related to their ascorbic acid status
Am. J. Clinical Nutrition,
January 1, 2005;
81(1):
95 - 103.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. Brigelius-Flohe, F. J Kelly, J. T Salonen, J. Neuzil, J.-M. Zingg, and A. Azzi
The European perspective on vitamin E: current knowledge and future research
Am. J. Clinical Nutrition,
October 1, 2002;
76(4):
703 - 716.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. Birringer, P. Pfluger, D. Kluth, N. Landes, and R. Brigelius-Flohe
Identities and Differences in the Metabolism of Tocotrienols and Tocopherols in HepG2 Cells
J. Nutr.,
October 1, 2002;
132(10):
3113 - 3118.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
Q. Jiang, S. Christen, M. K Shigenaga, and B. N Ames
{gamma}-Tocopherol, the major form of vitamin E in the US diet, deserves more attention
Am. J. Clinical Nutrition,
December 1, 2001;
74(6):
714 - 722.
[Abstract]
[Full Text]
|
 |
|
Copyright © 2000 by the American Society for Biochemistry and Molecular Biology.
|
Advertisement
Advertisement
|