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Cited by 49Metabolism and Genetic DisordersLysosomal Storage Disorders ResearchFatty Acid Research and Health

A mixture of oleic, erucic and conjugated linoleic acids modulates cerebrospinal fluid inflammatory markers and improve somatosensorial evoked potential in X-linked adrenoleukodystrophy female carriers

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Cappa, Marco, Bizzarri, Carla, Petroni, Anna, Carta, Gianfranca, Cordeddu, Lina, Valeriani, Massimiliano, Vollono, Catello, De Pasquale, Loredana +2 more·2012·Journal of Inherited Metabolic Disease
DOI 10.1007/s10545-011-9432-3PMC PMC3432215
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Abstract

X-linked adrenoleukodystrophy is a rare inherited demyelinating disorder characterized by an abnormal accumulation of very long chain fatty acids, mainly hexacosanoic acid (26:0), due to a mutation of the gene encoding for a peroxisomal membrane protein. The only available, and partially effective, therapeutic treatment consists of dietary intake of a 4:1 mixture of triolein and trierucin, called Lorenzo's oil (LO), targeted to inhibit the elongation of docosanoic acid (22:0) to 26:0. In this study we tested whether, besides inhibiting elongation, an enhancement of peroxisomal beta oxidation induced by conjugated linoleic acid (CLA), will improve somatosensory evoked potentials and modify inflammatory markers in adrenoleukodystrophy females carriers. We enrolled five heterozygous women. They received a mixture of LO (40 g/day) with CLA (5 g/day) for 2 months. The therapeutic efficacy was evaluated by the means of plasma levels of 26:0, 26:0/22:0 ratio, modification of cerebrospinal fluid (CSF) inflammatory markers and somatosensory evoked potentials. Changes of fatty acid profile, and in particular CLA incorporation, were also evaluated in CSF and plasma. The results showed that CLA promptly passes the blood brain barrier and the mixture was able to lower both 26:0 and 26:0/22:0 ratio in plasma. The mixture improved somatosensory evoked potentials, which were previously found unchanged or worsened with dietary LO alone, and reduced IL-6 levels in CSF in three out of five patients. Our data suggest that the synergic activity of CLA and LO, by enhancing peroxisomal beta-oxidation and preventing 26:0 formation, improves the somatosensory evoked potentials and reduces neuroinflammation.

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  • A new oil mixture allows erucic acid to cross the blood brain barrier and reduce VLCFA levels in spinal fluid.

    “Recently, a new nutritional approach has been proposed with a new oil mixture allowing erucic acid to cross the blood brain barrier and reduce VLCFA levels in spinal fluid (45).”

    From X-linked adrenoleukodystrophy and primary adrenal insufficiency (2023) · cites this paper as [45]

    The Results (Lipid metabolism findings) report that the LO + CLA mixture lowered VLCFA measures in plasma, but VLCFAs and erucic acid were below detectable levels in cerebrospinal fluid, so a reduction there was not shown. The Introduction says erucic acid likely crosses the blood–brain barrier, but the paper does not show that the new mixture enables it to do so; it reports that CLA crosses instead. (LLM, conf=0.88)

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Cited by

Sorted by citations

Authors
Cappa, M., Bizzarri, C., Petroni, A., Carta, G., Cordeddu, L., Valeriani, M., Vollono, C., De Pasquale, L., Blasevich, M., & Banni, S.
Affiliations
  • Bambino Gesù Children's Hospital (IT)
  • University of Milan (IT)
  • University of Cagliari (IT)
  • Laboratori di Ricerca Ospedale Pediatrico Bambino Gesù Roma Italy
Corresponding author
Banni, Sebastiano
ORCID
Venue
Journal of Inherited Metabolic Disease
Publisher
Wiley
Published
2011-12-22
Volume
35
Issue
5
Pages
899-907
ISSN
  • 0141-8955 (ISSN-L)
  • 1573-2665
DOI
10.1007/s10545-011-9432-3
PMC
PMC3432215
OpenAlex
W2032127985
Cited by
49
References
2
Citations by year
2026: 2 · 2025: 2 · 2024: 6 · 2023: 7 · 2022: 3 · 2021: 5 · 2020: 3 · 2019: 3 · 2018: 4 · 2017: 3 · 2016: 1 · 2015: 2 · 2014: 2 · 2013: 3 · 2012: 1
FWCI
1.16
Type
journal article
Language
en
Open access
Licence
Locations
  • Journal of Inherited Metabolic Disease (publishedVersion, cc-by-nc)
  • Europe PMC (PubMed Central) (publishedVersion, cc-by-nc)
  • PubMed Central (publishedVersion, cc-by-nc)
  • PubMed (publishedVersion)
  • Cineca Institutional Research Information System (Tor Vergata University) (submittedVersion)
  • UNICA IRIS Institutional Research Information System (University of Cagliari) (submittedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
  • Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
  • Health Sciences › Nursing › Nutrition and Dietetics › Fatty Acid Research and Health
Keywords
X-linked adrenoleukodystrophy, Lorenzo's oil, conjugated linoleic acid, oleic acid, erucic acid, very long chain fatty acids, hexacosanoic acid, peroxisomal beta-oxidation, somatosensory evoked potentials, interleukin-6, neuroinflammation, female carriers
MeSH terms
Adrenoleukodystrophy, Blood-Brain Barrier, Drug Combinations, Erucic Acids, Evoked Potentials, Somatosensory, Fatty Acids, Female, Heterozygote, Humans, Inflammation, Middle Aged, Oxidation-Reduction, Triolein, Biomarkers, Interleukin-6, Inflammation Mediators, Oleic Acid, Linoleic Acids, Conjugated, Lipid Metabolism

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