Literature DB >> 7205256

Abnormality of long-chain fatty acids in erythrocyte membrane sphingomyelin from patients with adrenoleukodystrophy.

S Tsuji, M Suzuki, T Ariga, M Sekine, M Kuriyama, T Miyatake.   

Abstract

We have devised an analytical method for the determination of fatty acid composition of erythrocyte membrane sphingomyelin by chemical ionization mass spectrometry combined with capillary column gas-liquid chromatography. Fatty acid composition of erythrocyte membrane sphingomyelin from 8 patients with adrenoleukodystrophy (ALD) and 16 healthy controls were examined by this method. The ratio of hexacosanoic acid (C26:0) to docosanoic acid (C22:0) in erythrocyte membrane sphingomyelin from ALD patients was 2.6-fold higher than that of the controls. This result suggests that biochemical diagnosis of ALD is possible by the analysis of fatty acid composition of erythrocyte membrane sphingomyelin. Furthermore, it demonstrates that biochemical abnormality in ALD is the generalized abnormal metabolism of very long-chain saturated fatty acids.

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Year:  1981        PMID： 7205256     DOI： 10.1111/j.1471-4159.1981.tb01698.x

Source DB:  PubMed          Journal:  J Neurochem        ISSN： 0022-3042            Impact factor:   5.372


  16 in total

Review 1.  X linked adrenoleukodystrophy: clinical presentation, diagnosis, and therapy.

Authors:  B M van Geel; J Assies; R J Wanders; P G Barth
Journal:  J Neurol Neurosurg Psychiatry       Date:  1997-07       Impact factor: 10.154

Review 2.  Evaluation of therapy of X-linked adrenoleukodystrophy.

Authors:  Hugo W Moser; Ali Fatemi; Kathleen Zackowski; Seth Smith; Xavier Golay; Larry Muenz; Gerald Raymond
Journal:  Neurochem Res       Date:  2004-05       Impact factor: 3.996

3.  A family with adrenoleucodystrophy.

Authors:  R P Singh; S Deshpande; R K Marwaha; K Garg
Journal:  Indian J Pediatr       Date:  1989 Sep-Oct       Impact factor: 1.967

4.  Adrenoleukodystrophy without adrenal insufficiency and its magnetic resonance imaging.

Authors:  H Nishio; S Kodama; T Tsubota; T Takumi; T Takahashi; S Yokoyama; T Matsuo
Journal:  J Neurol       Date:  1985       Impact factor: 4.849

5.  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

6.  Membrane microviscosity is increased in the erythrocytes of patients with adrenoleukodystrophy and adrenomyeloneuropathy.

Authors:  R A Knazek; W B Rizzo; J D Schulman; J R Dave
Journal:  J Clin Invest       Date:  1983-07       Impact factor: 14.808

7.  Novel subtype of peroxisomal acyl-CoA oxidase deficiency and bifunctional enzyme deficiency with detectable enzyme protein: identification by means of complementation analysis.

Authors:  Y Suzuki; N Shimozawa; S Yajima; S Tomatsu; N Kondo; Y Nakada; S Akaboshi; M Lai; Y Tanabe; T Hashimoto
Journal:  Am J Hum Genet       Date:  1994-01       Impact factor: 11.025

8.  Adrenoleukodystrophy: a clinical variant presenting as olivopontocerebellar atrophy.

Authors:  T Ohno; H Tsuchida; N Fukuhara; T Yuasa; H Harayama; S Tsuji; T Miyatake
Journal:  J Neurol       Date:  1984       Impact factor: 4.849

9.  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 10.  Very long chain fatty acids in higher animals--a review.

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