Literature DB >> 7172439

Detection of adrenoleukodystrophy by increased C26:0 fatty acid levels in leukocytes.

B Molzer, H Bernheimer, R Heller, K Toifl, M Vetterlein.   

Abstract

Very long chain fatty acids of peripheral blood leukocytes were analyzed by gas chromatography in nine members of a family including two hemizygotes and one obligate heterozygote for adrenoleukodystrophy (ALD), as well as in twelve controls. Comparative investigations were done in cultured fibroblasts. Elevated C26:0 levels were observed in leukocytes and fibroblasts of ALD hemizygotes. The obligate heterozygote displayed a clear-cut increase of C26:0 concentration in leukocytes but not in fibroblasts. Determination of C26:0 in leukocytes may serve as diagnostic tool in the detection of ALD gene carriers.

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Year:  1982        PMID: 7172439     DOI: 10.1016/0009-8981(82)90260-1

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


  7 in total

Review 1.  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

2.  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

3.  Determination of C20-C30 fatty acids by reversed-phase chromatographic techniques: an efficient method to quantitate minor fatty acids in serum of patients with adrenoleukodystrophy.

Authors:  M Alberghina; A Fiumara; L Pavone; A M Giuffrida
Journal:  Neurochem Res       Date:  1984-12       Impact factor: 3.996

4.  Adrenoleukodystrophy: detection of increased very long chain fatty acids by high-performance liquid chromatography.

Authors:  T Kobayashi; M Katayama; S Suzuki; H Tomoda; I Goto; Y Kuroiwa
Journal:  J Neurol       Date:  1983       Impact factor: 4.849

Review 5.  Very long chain fatty acids in higher animals--a review.

Authors:  A Poulos
Journal:  Lipids       Date:  1995-01       Impact factor: 1.880

6.  Adrenoleukodystrophy in an adult female. A clinical, morphological, and neurochemical study.

Authors:  W Schlote; B Molzer; J Peiffer; M Poremba; F Schumm; K Harzer; R Schnabel; H Bernheimer
Journal:  J Neurol       Date:  1987-10       Impact factor: 4.849

7.  Brain tissue immunoglobulins in adrenoleukodystrophy: a comparison with multiple sclerosis and systemic lupus erythematosus.

Authors:  H Bernheimer; H Budka; P Müller
Journal:  Acta Neuropathol       Date:  1983       Impact factor: 17.088

  7 in total

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