Literature DB >> 7800117

Brain, liver, and adipose tissue erucic and very long chain fatty acid levels in adrenoleukodystrophy patients treated with glyceryl trierucate and trioleate oils (Lorenzo's oil).

M Rasmussen1, A B Moser, J Borel, S Khangoora, H W Moser.   

Abstract

Brain, liver, and adipose lipids were studied in the postmortem tissues of four adrenoleukodystrophy patients who had been treated with a mixture of glyceryl trioleate and trierucate oils ("Lorenzo's Oil") and compared to 7 untreated ALD patients and 3 controls. The dietary therapy appeared to reduce the levels of saturated very long chain fatty acids in the plasma, adipose tissue and liver; in the brain they were reduced in only one of the four patients. While substantial amounts of erucic acid were present in some of the tissues even 12 months after therapy had been discontinued, the levels in brain did not exceed those in controls at any time. The failure of erucic acid to enter the brain in significant quantity may be a factor in the disappointing results of dietary therapy for adrenoleukodystrophy.

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Year:  1994        PMID: 7800117     DOI: 10.1007/bf00968719

Source DB:  PubMed          Journal:  Neurochem Res        ISSN: 0364-3190            Impact factor:   3.996


  31 in total

1.  Lorenzo's oil.

Authors:  H W Moser
Journal:  Lancet       Date:  1993-02-27       Impact factor: 79.321

2.  Nervonic acid biosynthesis by erucyl-CoA elongation in normal and quaking mouse brain microsomes. Elongation of other unsaturated fatty acyl-CoAs (mono and poly-unsaturated).

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Authors:  C J Reinecke; D P Knoll; P J Pretorius; H S Steyn; R H Simpson
Journal:  J Neurol Sci       Date:  1985-08       Impact factor: 3.181

5.  An adult case of adrenoleukodystrophy with features of olivo-ponto-cerebellar atrophy: II. Lipid biochemical studies.

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Journal:  Jpn J Exp Med       Date:  1987-02

6.  Metabolism of [17,18-3H2]hexacosanoic acid and [15,16-3H2]lignoceric acid in cultured skin fibroblasts from patients with adrenoleukodystrophy (ALD) and adrenomyeloneuropathy (AMN).

Authors:  S Tsuji; T Sano-Kawamura; T Ariga; T Miyatake
Journal:  J Neurol Sci       Date:  1985-12       Impact factor: 3.181

7.  A two-year trial of oleic and erucic acids ("Lorenzo's oil") as treatment for adrenomyeloneuropathy.

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Journal:  N Engl J Med       Date:  1993-09-09       Impact factor: 91.245

8.  Incorporation of dietary [14C]arachidonic acid and [3H]eicosapentaenoic acid into tissue lipids during absorption of a fish oil emulsion.

Authors:  A Nilsson; L Hjelte; B Strandvik
Journal:  J Lipid Res       Date:  1992-09       Impact factor: 5.922

9.  Quantitative determination of the neutral glycosyl ceramides in human blood.

Authors:  D E Vance; C C Sweeley
Journal:  J Lipid Res       Date:  1967-11       Impact factor: 5.922

10.  Phospholipids in X-linked adrenoleukodystrophy white matter: fatty acid abnormalities before the onset of demyelination.

Authors:  C Theda; A B Moser; J M Powers; H W Moser
Journal:  J Neurol Sci       Date:  1992-07       Impact factor: 3.181

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  18 in total

1.  Progression of abnormalities in adrenomyeloneuropathy and neurologically asymptomatic X-linked adrenoleukodystrophy despite treatment with "Lorenzo's oil".

Authors:  B M van Geel; J Assies; E B Haverkort; J H Koelman; B Verbeeten; R J Wanders; P G Barth
Journal:  J Neurol Neurosurg Psychiatry       Date:  1999-09       Impact factor: 10.154

Review 2.  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 3.  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

4.  A mouse model for X-linked adrenoleukodystrophy.

Authors:  J F Lu; A M Lawler; P A Watkins; J M Powers; A B Moser; H W Moser; K D Smith
Journal:  Proc Natl Acad Sci U S A       Date:  1997-08-19       Impact factor: 11.205

5.  Effects of exogenous hexacosanoic acid on biochemical myelin composition in weaning and post-weaning rats.

Authors:  A Di Biase; C Avellino; F Pieroni; T Quaresima; A Grisolia; M Cappa; S Salvati
Journal:  Neurochem Res       Date:  1997-03       Impact factor: 3.996

6.  A zebrafish model of X-linked adrenoleukodystrophy recapitulates key disease features and demonstrates a developmental requirement for abcd1 in oligodendrocyte patterning and myelination.

Authors:  Lauren R Strachan; Tamara J Stevenson; Briana Freshner; Matthew D Keefe; D Miranda Bowles; Joshua L Bonkowsky
Journal:  Hum Mol Genet       Date:  2017-09-15       Impact factor: 6.150

7.  Polyunsaturated fatty acid status in treated isovaleric acidemia patients.

Authors:  M Dercksen; W Kulik; L J Mienie; C J Reinecke; R J A Wanders; M Duran
Journal:  Eur J Clin Nutr       Date:  2016-06-22       Impact factor: 4.016

8.  Metabolism of trideuterated iso-lignoceric acid in rats in vivo and in human fibroblasts in culture.

Authors:  A Poulos; P C Stockham; D W Johnson; B C Paton; K Beckman; H Singh
Journal:  Lipids       Date:  1999-09       Impact factor: 1.880

9.  Contiguous deletion of the X-linked adrenoleukodystrophy gene (ABCD1) and DXS1357E: a novel neonatal phenotype similar to peroxisomal biogenesis disorders.

Authors:  Deyanira Corzo; William Gibson; Kisha Johnson; Grant Mitchell; Guy LePage; Gerald F Cox; Robin Casey; Carolyn Zeiss; Heidi Tyson; Garry R Cutting; Gerald V Raymond; Kirby D Smith; Paul A Watkins; Ann B Moser; Hugo W Moser; Steven J Steinberg
Journal:  Am J Hum Genet       Date:  2002-04-29       Impact factor: 11.025

10.  Erucic acid is differentially taken up and metabolized in rat liver and heart.

Authors:  Cameron C Murphy; Eric J Murphy; Mikhail Y Golovko
Journal:  Lipids       Date:  2008-03-19       Impact factor: 1.880

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