Cited by 46Metabolism and Genetic DisordersLipid metabolism and biosynthesisNeurological diseases and metabolism

Role of peroxisomes in human lipid metabolism and its importance for neurological development

Wanders, Ronald J.A., Poll-The, Bwee Tien·2017·Neuroscience Letters

Abstract

Peroxisomes play a crucial role in normal neurological development as exemplified by the devastating neurological consequences of a defect in the biogenesis of peroxisomes as in Zellweger syndrome. The underlying basis for the important role of peroxisomes in neurological development resides in the fact that peroxisomes catalyze a number of physiological functions, notably involving the metabolism of different lipids. Indeed, peroxisomes catalyse the beta-oxidative breakdown of certain fatty acids including: (1.) the very long-chain fatty acids C22:0, C24:0, and C26:0; (2.) pristanic acid and (3.) the bile acid intermediates di- and trihydroxycholestanoic acid which cannot be oxidized in mitochondria. Furthermore, peroxisomes catalyze the synthesis of a particular type of lipids, i.e. ether-linked phospholipids, which are highly abundant in brain, especially in myelin. The current state of knowledge with respect to the metabolic role of peroxisomes will be described in this paper with particular emphasis on the role of peroxisomes in brain.

Integrity signals

Registry

Crossref · 10.1016/j.neulet.2015.06.018

Cites

66 of 66 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

  • Most patients with acyl-CoA oxidase deficiency show neonatal onset of hypotonia, seizures, failure to thrive, hepatomegaly, psychomotor retardation, sensory deafness, absent reflexes, and visual loss with retinopathy and extinguished electroretinograms.

    “Most patients show neonatal onset of hypotonia, seizures, failure to thrive, hepatomegaly, psychomotor retardation, sensory deafness, absent reflexes, and visual loss with retinopathy and extinguished electroretinograms [80].”

    From Peroxisomes in brain development and function (2016) · cites this paper as [80]

  • Deficiency in FAR1 causes RCDP type 4.

    “the disorder of three patients with a deficiency in FAR1, the gene coding for fatty acyl-CoA reductase 1, which generates the fatty alcohols necessary to form the ether bond of the 1-alkyl chain in ether phospholipid biosynthesis [183], was categorized as RCDP type 4 [80].”

    From Peroxisomes in brain development and function (2016) · cites this paper as [80]

  • Two additional subtypes of RCDP were identified based on strong reduction of plasmalogen levels and similarity of symptoms with classical RCDP.

    “Recently, two additional subtypes of RCDP were identified based on the strong reduction of plasmalogen levels in the patients and the similarity of their symptoms with “classical” RCDP.”

    From Peroxisomes in brain development and function (2016) · cites this paper as [80]

Cites

Flagged first, then by citations

Cited by

Sorted by citations

Authors
Wanders, R. J. A., & Poll-The, B. T.
Affiliations
Amsterdam UMC Location University of Amsterdam (NL)
Corresponding author
Wanders, Ronald J.A.
Venue
Neuroscience Letters
Publisher
Elsevier BV
Published
2017-01
Volume
637
Pages
11-17
ISSN
0304-3940 (ISSN-L)
OpenAlex
W607611259
Cited by
46
References
66
Citations by year
2026: 1 · 2025: 1 · 2024: 7 · 2023: 5 · 2022: 3 · 2021: 5 · 2020: 3 · 2019: 6 · 2018: 4 · 2017: 5 · 2016: 3 · 2015: 3
FWCI
4.11
Type
journal article
Language
en
Open access
closed
Locations
  • Neuroscience Letters (publishedVersion)
  • PubMed (publishedVersion)
  • Pure Amsterdam UMC (publishedVersion)
  • Data Archiving and Networked Services (DANS) (submittedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Lipid metabolism and biosynthesis
  • Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
Keywords
peroxisomes, peroxisome biogenesis disorders, Zellweger syndrome, lipid metabolism, brain development, very long chain fatty acids, fatty acid beta-oxidation, pristanic acid, bile acid intermediates, ether phospholipids, myelin, brain
MeSH terms
Brain, Fatty Acids, Humans, Mitochondria, Oxidation-Reduction, Zellweger Syndrome, Peroxisomes, Lipid Metabolism

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review