Literature DB >> 3951702

Adrenoleukodystrophy: oleic acid lowers fibroblast saturated C22-26 fatty acids.

W B Rizzo, P A Watkins, M W Phillips, D Cranin, B Campbell, J Avigan.   

Abstract

Monounsaturated fatty acids, especially oleic acid (C18:1), decreased the content of saturated very-long-chain fatty acids (VLFA) in cultured skin fibroblasts from patients with adrenoleukodystrophy (ALD) and controls. When confluent ALD fibroblasts were incubated with oleic acid for 5 days in lipid-free medium, which eliminates uptake of exogenous VLFA, the mean cell content of C26:0 was decreased by 33.7 +/- 10.1%. Oleic acid inhibited C26:0 synthesis in ALD fibroblasts by 58% and total fatty acid synthesis by 68 to 78%. Therefore, the elevated C26:0 levels in ALD cells may be lowered by inhibiting fatty acid biosynthesis, and inhibition of saturated VLFA synthesis by oleate may be useful in treating ALD.

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Year:  1986        PMID： 3951702     DOI： 10.1212/wnl.36.3.357

Source DB:  PubMed          Journal:  Neurology        ISSN： 0028-3878            Impact factor:   9.910


  28 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

Review 5.  Peroxisomal disorders: clinical characterization.

Authors:  L Monnens; H Heymans
Journal:  J Inherit Metab Dis       Date:  1987       Impact factor: 4.982

Review 6.  Prenatal and perinatal diagnosis of peroxisomal disorders.

Authors:  R B Schutgens; G Schrakamp; R J Wanders; H S Heymans; J M Tager; H van den Bosch
Journal:  J Inherit Metab Dis       Date:  1989       Impact factor: 4.982

7.  Metabolic rerouting via SCD1 induction impacts X-linked adrenoleukodystrophy.

Authors:  Quentin Raas; Malu-Clair van de Beek; Sonja Forss-Petter; Inge Me Dijkstra; Abigail Deschiffart; Briana C Freshner; Tamara J Stevenson; Yorrick Rj Jaspers; Liselotte Nagtzaam; Ronald Ja Wanders; Michel van Weeghel; Joo-Yeon Engelen-Lee; Marc Engelen; Florian Eichler; Johannes Berger; Joshua L Bonkowsky; Stephan Kemp
Journal:  J Clin Invest       Date:  2021-04-15       Impact factor: 14.808

8.  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).

Authors:  M Rasmussen; A B Moser; J Borel; S Khangoora; H W Moser
Journal:  Neurochem Res       Date:  1994-08       Impact factor: 3.996

Review 9.  Adrenoleukodystrophy: phenotypic variability and implications for therapy.

Authors:  H W Moser; A B Moser; K D Smith; A Bergin; J Borel; J Shankroff; O C Stine; C Merette; J Ott; W Krivit
Journal:  J Inherit Metab Dis       Date:  1992       Impact factor: 4.982

10.  Monoenoic fatty acids in human brain lipids: isomer identification and distribution.

Authors:  D W Johnson; K Beckman; A J Fellenberg; B S Robinson; A Poulos
Journal:  Lipids       Date:  1992-03       Impact factor: 1.880
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