Literature DB >> 6723067

Serum very long chain fatty acid pattern in Zellweger syndrome.

J A Bakkeren, L A Monnens, J M Trijbels, J M Maas.   

Abstract

Very long chain fatty acids of serum were analyzed by gas chromatography in six patients with the cerebro-hepato-renal syndrome of Zellweger, in seven of their parents, and in ten normal children. Increased concentrations of hexacosanoic acid (C26:0) were consistently found in the Zellweger patients. The ratios C26:0/C22:0, C25:0/C22:0, and C24:0/C22:0 also were elevated. These findings can serve as an additional important diagnostic criterion for Zellweger syndrome. The very long chain fatty acid levels and ratios in the blood of the patients' parents were within the normal ranges.

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Year:  1984        PMID： 6723067     DOI： 10.1016/0009-8981(84)90140-2

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


  16 in total

1.  Linkage analysis in X-linked adrenoleukodystrophy and application in post- and prenatal diagnosis.

Authors:  B A van Oost; P M van Zandvoort; W Tünte; H G Brunner; A J Hoogeboom; P D Maaswinkel-Mooy; J Bakkeren; B Hamel; H H Ropers
Journal:  Hum Genet       Date:  1991-02       Impact factor: 4.132

Review 2.  The inborn errors of peroxisomal beta-oxidation: a review.

Authors:  R J Wanders; C W van Roermund; R B Schutgens; P G Barth; H S Heymans; H van den Bosch; J M Tager
Journal:  J Inherit Metab Dis       Date:  1990       Impact factor: 4.982

Review 3.  Meibomian glands, meibum, and meibogenesis.

Authors:  Igor A Butovich
Journal:  Exp Eye Res       Date:  2017-06-29       Impact factor: 3.467

4.  Neonatal adrenoleukodystrophy.

Authors:  P Aubourg; J Scotto; F Rocchiccioli; D Feldmann-Pautrat; O Robain
Journal:  J Neurol Neurosurg Psychiatry       Date:  1986-01       Impact factor: 10.154

Review 5.  Peroxisomal disorders: a newly recognised group of genetic diseases.

Authors:  R B Schutgens; H S Heymans; R J Wanders; H van den Bosch; J M Tager
Journal:  Eur J Pediatr       Date:  1986-02       Impact factor: 3.183

Review 6.  Very-long-chain fatty acids from lower organism.

Authors:  T Rezanka; J Cudlín; M Podojil
Journal:  Folia Microbiol (Praha)       Date:  1987       Impact factor: 2.099

7.  Disturbed adrenocortical function in cerebro-hepato-renal syndrome of Zellweger.

Authors:  L Govaerts; L Monnens; T Melis; F Trijbels
Journal:  Eur J Pediatr       Date:  1984-11       Impact factor: 3.183

8.  Presence of the peroxisomal 22-kDa integral membrane protein in the liver of a person lacking recognizable peroxisomes (Zellweger syndrome).

Authors:  P B Lazarow; Y Fujiki; G M Small; P Watkins; H Moser
Journal:  Proc Natl Acad Sci U S A       Date:  1986-12       Impact factor: 11.205

Review 9.  X-linked adrenoleukodystrophy: biochemical diagnosis and enzyme defect.

Authors:  R J Wanders; C W van Roermund; W Lageweg; B S Jakobs; R B Schutgens; A A Nijenhuis; J M Tager
Journal:  J Inherit Metab Dis       Date:  1992       Impact factor: 4.982

10.  Metabolism of prostaglandin F2 alpha in Zellweger syndrome. Peroxisomal beta-oxidation is a major importance for in vivo degradation of prostaglandins in humans.

Authors:  U Diczfalusy; B F Kase; S E Alexson; I Björkhem
Journal:  J Clin Invest       Date:  1991-09       Impact factor: 14.808
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