Literature DB >> 2440622

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.   

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.

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Year:  1987        PMID： 2440622     DOI： 10.1016/0009-8981(87)90193-8

Source DB:  PubMed          Journal:  Clin Chim Acta        ISSN： 0009-8981            Impact factor:   3.786


  7 in total

1.  Plasmalogens and oxidative stress: evidence against a major role of plasmalogens in protection against the superoxide anion radical.

Authors:  G A Jansen; R J Wanders
Journal:  J Inherit Metab Dis       Date:  1997-03       Impact factor: 4.982

Review 2.  Inherited peroxisomal disorders involving the nervous system.

Authors:  J B Stephenson
Journal:  Arch Dis Child       Date:  1988-07       Impact factor: 3.791

3.  Rhizomelic chondrodysplasia punctata. Deficiency of 3-oxoacyl-coenzyme A thiolase in peroxisomes and impaired processing of the enzyme.

Authors:  J C Heikoop; C W van Roermund; W W Just; R Ofman; R B Schutgens; H S Heymans; R J Wanders; J M Tager
Journal:  J Clin Invest       Date:  1990-07       Impact factor: 14.808

Review 4.  Plasmalogens and fatty alcohols in rhizomelic chondrodysplasia punctata and Sjögren-Larsson syndrome.

Authors:  Ana R Malheiro; Tiago Ferreira da Silva; Pedro Brites
Journal:  J Inherit Metab Dis       Date:  2014-11-29       Impact factor: 4.982

5.  Peroxisomal fatty acid beta-oxidation in relation to the accumulation of very long chain fatty acids in cultured skin fibroblasts from patients with Zellweger syndrome and other peroxisomal disorders.

Authors:  R J Wanders; C W van Roermund; M J van Wijland; R B Schutgens; J Heikoop; H van den Bosch; A W Schram; J M Tager
Journal:  J Clin Invest       Date:  1987-12       Impact factor: 14.808

6.  Genetic heterogeneity in the cerebrohepatorenal (Zellweger) syndrome and other inherited disorders with a generalized impairment of peroxisomal functions. A study using complementation analysis.

Authors:  S Brul; A Westerveld; A Strijland; R J Wanders; A W Schram; H S Heymans; R B Schutgens; H van den Bosch; J M Tager
Journal:  J Clin Invest       Date:  1988-06       Impact factor: 14.808

7.  Studies on the oxidation of phytanic acid and pristanic acid in human fibroblasts by acylcarnitine analysis.

Authors:  N M Verhoeven; C Jakobs; H J ten Brink; R J Wanders; C R Roe
Journal:  J Inherit Metab Dis       Date:  1998-10       Impact factor: 4.982
  7 in total

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