Evidence map›Paper›PMID 35378685›Full record

ArticleNeurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics2022

Nervonic Acid Attenuates Accumulation of Very Long-Chain Fatty Acids and is a Potential Therapy for Adrenoleukodystrophy.

Marcia R Terluk, Julianne Tieu, Siddhee A Sahasrabudhe, Ann Moser, Paul A Watkins, Gerald V Raymond, Reena V Kartha

Open access · bronzeAbstract read
In one paragraph

Article in Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.

0numbers the graph read from it
0cells of the map it votes in
13citing papers in PubMed
2.5field-weighted citation impact, top 10% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

13 citing papers in PubMed, 30 citations in OpenAlex.

  1. The chromosome-level genomes of twoHorticulture research · 2026
    Article
  2. Nervonic acid supplementation mitigates disease severity biomarkers in adrenoleukodystrophy.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2026
    Article
  3. Review
  4. Article
  5. Article
  6. Article
  7. Review
  8. Erucic acid utilization byFrontiers in microbiology · 2024
    Article
  9. Article
  10. Review
  11. Review
  12. Article
  13. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

7 authors at 3 institutions in 1 country.

Marcia R TerlukCenter for Orphan Drug Research, University of Minnesota, McGuire Translational Research Facility, 2001 6th St SE, Minneapolis, MN, 55455, USA.
Julianne TieuCenter for Orphan Drug Research, University of Minnesota, McGuire Translational Research Facility, 2001 6th St SE, Minneapolis, MN, 55455, USA.
Siddhee A SahasrabudheCenter for Orphan Drug Research, University of Minnesota, McGuire Translational Research Facility, 2001 6th St SE, Minneapolis, MN, 55455, USA.
Ann MoserHugo W. Moser Research Institute at Kennedy Krieger, Baltimore, MD, 21205, USA.
Paul A WatkinsHugo W. Moser Research Institute at Kennedy Krieger, Baltimore, MD, 21205, USA.
Gerald V RaymondDepartment of Neurology, John Hopkins University, Baltimore, MD, 21287, USA.
Reena V KarthaCenter for Orphan Drug Research, University of Minnesota, McGuire Translational Research Facility, 2001 6th St SE, Minneapolis, MN, 55455, USA. rvkartha@umn.edu.ORCID 0000-0002-1692-1539
University of Minnesota · USKennedy Krieger Institute · USJohns Hopkins University · US

Funding

Dose Optimization of Nervonic Acid - a Potential Therapy to Alleviate Disease Progression in AdrenoleukodystrophyR21TR003941 · NCATS · UNIVERSITY OF MINNESOTA · PI KARTHA, REENA V · 2021 to 2022
$426k
NCATS NIH HHS R21 TR003941
6 · The paper itself

Abstract

Adrenoleukodystrophy (ALD) is an X-linked inherited peroxisomal disorder due to mutations in the ALD protein and characterized by accumulation of very long-chain fatty acids (VLCFA), specifically hexacosanoic acid (C26:0). This can trigger other pathological processes such as mitochondrial dysfunction, oxidative stress, and inflammation, which if involves the brain tissues can result in a lethal form of the disease called childhood cerebral ALD. With the recent addition of ALD to the Recommended Uniform Screening Panel, there is an increase in the number of individuals who are identified with ALD. However, currently, there is no approved treatment for pre-symptomatic individuals that can arrest or delay symptom development. Here, we report our observations investigating nervonic acid, a monounsaturated fatty acid as a potential therapy for ALD. Using ALD patient-derived fibroblasts, we examined whether nervonic acid can reverse VLCFA accumulation similar to erucic acid, the active ingredient in Lorenzo's oil, a dietary intervention believed to alter disease course. We have shown that nervonic acid can reverse total lipid C26:0 accumulation in a concentration-dependent manner in ALD cell lines. Further, we show that nervonic acid can protect ALD fibroblasts from oxidative insults, presumably by increasing intracellular ATP production. Thus, nervonic acid can be a potential therapeutic for individuals with ALD, which can alter cellular biochemistry and improve its function.

Indexed as

AdrenoleukodystrophyChildFatty AcidsFatty Acids, MonounsaturatedFibroblastsHumansFatty AcidsFatty Acids, Monounsaturatednervonic acidAdrenoleukodystrophyDietary lipidsFibroblastsMonounsaturated fatty acidsPeroxisomesSphingomyelinVery long-chain fatty acids

Identifiers

PMID35378685
PMCPMC9294126
OpenAlexW4226102337

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.