Literature DB >> 6198480

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

T Kobayashi, M Katayama, S Suzuki, H Tomoda, I Goto, Y Kuroiwa.   

Abstract

Using high-performance liquid chromatography, adrenoleukodystrophy (ALD) and adrenomyeloneuropathy (AMN) were diagnosed by the analysis of fatty acids in sphingomyelin. The analytical method was simple, sensitive and accurate. In ALD and AMN patients, very long chain fatty acids (C26:0, C25:0 and C24:0 on the base of C22:0) were elevated not only in plasma and erythrocyte membranes but also in whole blood samples. An increase of long chain fatty acids was also shown in sphingomyelin of cultured skin fibroblasts. One heterozygote for ALD showed intermediate values between homozygotes and controls. Thus, high-performance liquid chromatography is a valuable method to detect increased long chain fatty acids for the diagnosis of ALD or AMN.

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Year:  1983        PMID： 6198480     DOI： 10.1007/bf00313696

Source DB:  PubMed          Journal:  J Neurol        ISSN： 0340-5354            Impact factor:   4.849


  15 in total

1.  The preparation and chemical characteristics of hemoglobin-free ghosts of human erythrocytes.

Authors:  J T DODGE; C MITCHELL; D J HANAHAN
Journal:  Arch Biochem Biophys       Date:  1963-01       Impact factor: 4.013

2.  Adrenoleukodystrophy. A clinical and pathological study of 17 cases.

Authors:  H H Schaumburg; J M Powers; C S Raine; K Suzuki; E P Richardson
Journal:  Arch Neurol       Date:  1975-09

3.  Adrenomyeloneuropathy: a probable variant of adrenoleukodystrophy. II. General pathologic, neuropathologic, and biochemical aspects.

Authors:  H H Schaumburg; J M Powers; C S Raine; P S Spencer; J W Griffin; J W Prineas; D M Boehme
Journal:  Neurology       Date:  1977-12       Impact factor: 9.910

4.  Adrenomyeloneuropathy: increased accumulation of very long chain fatty acid in cultured skeletal muscle.

Authors:  J McLaughlin; V Askanas; W K Engel
Journal:  Biochem Biophys Res Commun       Date:  1980-02-27       Impact factor: 3.575

5.  Assay for polyunsaturated fatty acids by argentation--thin-layer chromatography using commercial thin-layer plates.

Authors:  M Inomata; F Takaku; Y Nagai; M Saito
Journal:  Anal Biochem       Date:  1982-09-01       Impact factor: 3.365

6.  High concentration of hexacosanoate in cultured skin fibroblast lipids from adrenoleukodystrophy patients.

Authors:  N Kawamura; A B Moser; H W Moser; T Ogino; K Suzuki; H Schaumburg; A Milunsky; J Murphy; Y Kishimoto
Journal:  Biochem Biophys Res Commun       Date:  1978-05-15       Impact factor: 3.575

7.  Lipids and proteins of erythrocyte membrane in Duchenne muscular dystrophy.

Authors:  T Kobayashi; S Mawatari; Y Kuroiwa
Journal:  Clin Chim Acta       Date:  1978-05-02       Impact factor: 3.786

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

Authors:  B Molzer; H Bernheimer; R Heller; K Toifl; M Vetterlein
Journal:  Clin Chim Acta       Date:  1982-11-10       Impact factor: 3.786

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

Authors:  S Tsuji; M Suzuki; T Ariga; M Sekine; M Kuriyama; T Miyatake
Journal:  J Neurochem       Date:  1981-03       Impact factor: 5.372

10.  Adrenoleukodystrophy: increased plasma content of saturated very long chain fatty acids.

Authors:  H W Moser; A B Moser; K K Frayer; W Chen; J D Schulman; B P O'Neill; Y Kishimoto
Journal:  Neurology       Date:  1981-10       Impact factor: 9.910
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  4 in total

Review 1.  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

2.  Protease inhibitors suppress the degradation of mutant adrenoleukodystrophy proteins but do not correct impairment of very long chain fatty acid metabolism in adrenoleukodystrophy fibroblasts.

Authors:  T Yamada; N Shinnoh; T Kobayashi
Journal:  Neurochem Res       Date:  1997-03       Impact factor: 3.996

3.  Lovastatin does not correct the accumulation of very long-chain fatty acids in tissues of adrenoleukodystrophy protein-deficient mice.

Authors:  T Yamada; N Shinnoh; T Taniwaki; Y Ohyagi; H Asahara; J Kira
Journal:  J Inherit Metab Dis       Date:  2000-09       Impact factor: 4.982

4.  Familial spinocerebellar degeneration as an expression of adrenoleukodystrophy.

Authors:  T Kobayashi; S Noda; H Umezaki; I Goto; S Suzuki; T Kitaguchi; Y Kuroiwa
Journal:  J Neurol Neurosurg Psychiatry       Date:  1986-12       Impact factor: 10.154
  4 in total

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